Hair & Nails

ORLANDO – Platelet-rich plasma offers much for patients and dermatologists: It’s low-risk, has a low cost of entry, and usefully augments other medications and procedures for androgenetic alopecia and facial rejuvenation.

But there’s work to be done in standardizing its use and really understanding where, when, and for whom platelet-rich plasma (PRP) will be best used, said Dierdre Hooper, MD, a dermatologist in private practice in New Orleans.

As far back as the 1970s, PRP was used as a transfusion product, with use expanding during the following decade. “It’s really the ‘everywhere’ product,’” said Dr. Hooper, speaking at the Aesthetic, Surgical, and Clinical Dermatology Conference (ODAC).

Over the course of the past four decades, PRP has been explored for musculoskeletal healing, in gynecology, urology, cardiac surgery, ophthalmology, and for plastic surgery. “Initial skepticism has given way as some evidence is building,” said Dr. Hooper.

PRP, considered a biologic product, is produced by centrifuging a donor venipuncture. Among the pros of using PRP in a clinical practice, said Dr. Hooper, is the fact that numerous clinical studies do show benefit. The risk is low, as is the cost, and downtime is brief. All of these contribute to attractiveness to patients, who also like the idea of an all-natural product with an autologous source.

But consensus is lacking about some key aspects of utilization, including the best mode of preparation and optimal treatment schedule. Outcomes can be unpredictable, making it tough to say how cost-effective the regimen will be for a particular patient. “The ‘cons’ just come down to no consensus,” said Dr. Hooper.

Some of the basic science makes a compelling case for PRP: Activated platelets have secretory granules. These modify the pericellular milieu through release of a variety of growth factors by secretory granules. “We all were taught back in the day that platelets adhere to promote clotting, but they do a lot more than that – when the platelet is activated, it releases growth factors,” said Dr. Hooper. “Big picture? Think: This is how we heal.”

After blood collection, the sample is centrifuged. The goal of centrifuging is to achieve a platelet concentration of 1 to 1.5 million platelets per mL, or four to six times the platelet concentration seen in whole blood. In practice, there are variations in the mode of preparation, and in an individual’s platelet level at the time of venipuncture, said Dr. Hooper, so it’s hard to know what the platelet “dose” is from PRP.

After centrifuging, the sample will be stratified into a bottom portion, consisting primarily of red blood cells, a middle portion that’s the PRP, and a top portion that is platelet-poor plasma. Dr. Hooper draws up and saves the platelet- poor plasma as well, since it probably also contains some growth factors. She’ll save that for application or injection after a PRP treatment for some patients.

[email protected]

SOURCE: Hooper, D. ODAC 2018.

ORLANDO – Platelet-rich plasma offers much for patients and dermatologists: It’s low-risk, has a low cost of entry, and usefully augments other medications and procedures for androgenetic alopecia and facial rejuvenation.

But there’s work to be done in standardizing its use and really understanding where, when, and for whom platelet-rich plasma (PRP) will be best used, said Dierdre Hooper, MD, a dermatologist in private practice in New Orleans.

As far back as the 1970s, PRP was used as a transfusion product, with use expanding during the following decade. “It’s really the ‘everywhere’ product,’” said Dr. Hooper, speaking at the Aesthetic, Surgical, and Clinical Dermatology Conference (ODAC).

Over the course of the past four decades, PRP has been explored for musculoskeletal healing, in gynecology, urology, cardiac surgery, ophthalmology, and for plastic surgery. “Initial skepticism has given way as some evidence is building,” said Dr. Hooper.

PRP, considered a biologic product, is produced by centrifuging a donor venipuncture. Among the pros of using PRP in a clinical practice, said Dr. Hooper, is the fact that numerous clinical studies do show benefit. The risk is low, as is the cost, and downtime is brief. All of these contribute to attractiveness to patients, who also like the idea of an all-natural product with an autologous source.

But consensus is lacking about some key aspects of utilization, including the best mode of preparation and optimal treatment schedule. Outcomes can be unpredictable, making it tough to say how cost-effective the regimen will be for a particular patient. “The ‘cons’ just come down to no consensus,” said Dr. Hooper.

Some of the basic science makes a compelling case for PRP: Activated platelets have secretory granules. These modify the pericellular milieu through release of a variety of growth factors by secretory granules. “We all were taught back in the day that platelets adhere to promote clotting, but they do a lot more than that – when the platelet is activated, it releases growth factors,” said Dr. Hooper. “Big picture? Think: This is how we heal.”

After blood collection, the sample is centrifuged. The goal of centrifuging is to achieve a platelet concentration of 1 to 1.5 million platelets per mL, or four to six times the platelet concentration seen in whole blood. In practice, there are variations in the mode of preparation, and in an individual’s platelet level at the time of venipuncture, said Dr. Hooper, so it’s hard to know what the platelet “dose” is from PRP.

After centrifuging, the sample will be stratified into a bottom portion, consisting primarily of red blood cells, a middle portion that’s the PRP, and a top portion that is platelet-poor plasma. Dr. Hooper draws up and saves the platelet- poor plasma as well, since it probably also contains some growth factors. She’ll save that for application or injection after a PRP treatment for some patients.

[email protected]

SOURCE: Hooper, D. ODAC 2018.

ORLANDO – Platelet-rich plasma offers much for patients and dermatologists: It’s low-risk, has a low cost of entry, and usefully augments other medications and procedures for androgenetic alopecia and facial rejuvenation.

But there’s work to be done in standardizing its use and really understanding where, when, and for whom platelet-rich plasma (PRP) will be best used, said Dierdre Hooper, MD, a dermatologist in private practice in New Orleans.

As far back as the 1970s, PRP was used as a transfusion product, with use expanding during the following decade. “It’s really the ‘everywhere’ product,’” said Dr. Hooper, speaking at the Aesthetic, Surgical, and Clinical Dermatology Conference (ODAC).

Over the course of the past four decades, PRP has been explored for musculoskeletal healing, in gynecology, urology, cardiac surgery, ophthalmology, and for plastic surgery. “Initial skepticism has given way as some evidence is building,” said Dr. Hooper.

PRP, considered a biologic product, is produced by centrifuging a donor venipuncture. Among the pros of using PRP in a clinical practice, said Dr. Hooper, is the fact that numerous clinical studies do show benefit. The risk is low, as is the cost, and downtime is brief. All of these contribute to attractiveness to patients, who also like the idea of an all-natural product with an autologous source.

But consensus is lacking about some key aspects of utilization, including the best mode of preparation and optimal treatment schedule. Outcomes can be unpredictable, making it tough to say how cost-effective the regimen will be for a particular patient. “The ‘cons’ just come down to no consensus,” said Dr. Hooper.

Some of the basic science makes a compelling case for PRP: Activated platelets have secretory granules. These modify the pericellular milieu through release of a variety of growth factors by secretory granules. “We all were taught back in the day that platelets adhere to promote clotting, but they do a lot more than that – when the platelet is activated, it releases growth factors,” said Dr. Hooper. “Big picture? Think: This is how we heal.”

After blood collection, the sample is centrifuged. The goal of centrifuging is to achieve a platelet concentration of 1 to 1.5 million platelets per mL, or four to six times the platelet concentration seen in whole blood. In practice, there are variations in the mode of preparation, and in an individual’s platelet level at the time of venipuncture, said Dr. Hooper, so it’s hard to know what the platelet “dose” is from PRP.

After centrifuging, the sample will be stratified into a bottom portion, consisting primarily of red blood cells, a middle portion that’s the PRP, and a top portion that is platelet-poor plasma. Dr. Hooper draws up and saves the platelet- poor plasma as well, since it probably also contains some growth factors. She’ll save that for application or injection after a PRP treatment for some patients.

[email protected]

SOURCE: Hooper, D. ODAC 2018.

The Diagnosis: White Piedra

A fungal culture demonstrated a filamentous fungus that was identified as Trichosporon inkin via DNA sequencing, which confirmed the diagnosis of white piedra (WP).

Piedra refers to a group of fungal infections presenting as gritty nodules adherent to the hair shaft.¹ It is further categorized into black piedra, which occurs more commonly in tropical climates and is caused by Piedraia hortae, and WP, which occurs in tropical and temperate climates and is caused by the Trichosporon genus.^1-3 Among the Trichosporon genus, clinical manifestations have varied based on species; for example, T inkin commonly causes genital WP, Trichosporon ovoides commonly causes scalp WP, and Trichosporon asahii and Trichosporon mucoides have been described to cause systemic fungal infections in immunocompromised hosts.^1,4 Scalp WP most commonly occurs in children and young adults, and females are at greater risk than males.^1,2,5,6

Clinically, WP presents with pale irregular nodules along the hair shaft that are not fluorescent on Wood lamp examination.^1,6,7 Nodules are soft and easily detached from the hair shaft, unlike the hard, tightly adherent nodules seen in black piedra.^1,7 White piedra affects hair in a variety of areas including the scalp, beard, eyebrows, eyelashes, axillae, and genitals.^1,7 Affected hair may become brittle and break at points of invasion.1 Alternatively, WP may resemble tinea capitis with scalp hyperkeratosis and alopecia, though tinea typically affects the base of the hair shaft.¹ Immunocompromised patients can develop disseminated WP, and cases of progressive pneumonia, lung abscess, peritonitis, vascular access infection, and endocarditis have been reported.²

Diagnosis of WP is made through a combination of clinical findings and culture of infected hair. Potassium hydroxide preparation demonstrates sleevelike concretions formed of masses of septate hyphae with dense zones of arthrospores and blastospores.^1,2 Culture on Sabouraud agar demonstrates creamy colonies that develop a dull, gray, wrinkled surface.^1,2 Differential diagnosis includes pediculosis; however, the concretions of WP are circumferential around the hair shaft on microscopy.¹ Notably, a case of concomitant WP and pediculosis has been reported.⁸ In cases of potential pediculosis resistant to therapy, consider hair casts, which are asymptomatic, white, cylindrical concretions that encircle the hair without adherence and can therefore be differentiated from pediculosis via dermoscopy.⁹ Because this phenomenon is more commonly observed in preadolescent girls, it is hypothesized that scalp inflammation due to traction from hairstyles or atopic dermatitis contributes to the development of hair casts.^9,10 Thus, when a potassium hydroxide mount is equivocal for nits and dermoscopy demonstrates concretions that completely encircle the hair shaft, it is important to perform a microbiologic culture to rule out piedra of the hair or scalp. Other differential diagnoses include tinea capitis, black piedra, trichobacteriosis, and hair shaft abnormalities.

Transmission of WP is thought to result from a combination of poor hygiene; humidity due to climate; personal care practices such as habitually tying wet hair, applying hair oils and conditioners, or covering hair according to social customs; and close contact with an infected individual.^1,3,6 Long scalp hair potentially correlates with increased risk.^1,6 Finally, WP has been described in animals and has been isolated from soil, vegetable matter, and water.^3,10

Treatment of WP generally involves removal of infected hair, antifungal agents, and improved hygienic habits to avoid relapses. The American Academy of Dermatology’s Guidelines/Outcomes Committee recommends complete removal of infected hair; however, patients may desire hair-preserving treatments.¹¹ Kiken et al¹ reported success with the combination of an oral azole antifungal agent for 3 weeks to 1 month and an antifungal shampoo for 2 to 3 months. The authors proposed that oral medication eliminates scalp carriage while antifungal shampoo eliminates hair shaft concretions.¹
 

The Diagnosis: White Piedra

A fungal culture demonstrated a filamentous fungus that was identified as Trichosporon inkin via DNA sequencing, which confirmed the diagnosis of white piedra (WP).

Piedra refers to a group of fungal infections presenting as gritty nodules adherent to the hair shaft.¹ It is further categorized into black piedra, which occurs more commonly in tropical climates and is caused by Piedraia hortae, and WP, which occurs in tropical and temperate climates and is caused by the Trichosporon genus.^1-3 Among the Trichosporon genus, clinical manifestations have varied based on species; for example, T inkin commonly causes genital WP, Trichosporon ovoides commonly causes scalp WP, and Trichosporon asahii and Trichosporon mucoides have been described to cause systemic fungal infections in immunocompromised hosts.^1,4 Scalp WP most commonly occurs in children and young adults, and females are at greater risk than males.^1,2,5,6

Clinically, WP presents with pale irregular nodules along the hair shaft that are not fluorescent on Wood lamp examination.^1,6,7 Nodules are soft and easily detached from the hair shaft, unlike the hard, tightly adherent nodules seen in black piedra.^1,7 White piedra affects hair in a variety of areas including the scalp, beard, eyebrows, eyelashes, axillae, and genitals.^1,7 Affected hair may become brittle and break at points of invasion.1 Alternatively, WP may resemble tinea capitis with scalp hyperkeratosis and alopecia, though tinea typically affects the base of the hair shaft.¹ Immunocompromised patients can develop disseminated WP, and cases of progressive pneumonia, lung abscess, peritonitis, vascular access infection, and endocarditis have been reported.²

Diagnosis of WP is made through a combination of clinical findings and culture of infected hair. Potassium hydroxide preparation demonstrates sleevelike concretions formed of masses of septate hyphae with dense zones of arthrospores and blastospores.^1,2 Culture on Sabouraud agar demonstrates creamy colonies that develop a dull, gray, wrinkled surface.^1,2 Differential diagnosis includes pediculosis; however, the concretions of WP are circumferential around the hair shaft on microscopy.¹ Notably, a case of concomitant WP and pediculosis has been reported.⁸ In cases of potential pediculosis resistant to therapy, consider hair casts, which are asymptomatic, white, cylindrical concretions that encircle the hair without adherence and can therefore be differentiated from pediculosis via dermoscopy.⁹ Because this phenomenon is more commonly observed in preadolescent girls, it is hypothesized that scalp inflammation due to traction from hairstyles or atopic dermatitis contributes to the development of hair casts.^9,10 Thus, when a potassium hydroxide mount is equivocal for nits and dermoscopy demonstrates concretions that completely encircle the hair shaft, it is important to perform a microbiologic culture to rule out piedra of the hair or scalp. Other differential diagnoses include tinea capitis, black piedra, trichobacteriosis, and hair shaft abnormalities.

Transmission of WP is thought to result from a combination of poor hygiene; humidity due to climate; personal care practices such as habitually tying wet hair, applying hair oils and conditioners, or covering hair according to social customs; and close contact with an infected individual.^1,3,6 Long scalp hair potentially correlates with increased risk.^1,6 Finally, WP has been described in animals and has been isolated from soil, vegetable matter, and water.^3,10

Treatment of WP generally involves removal of infected hair, antifungal agents, and improved hygienic habits to avoid relapses. The American Academy of Dermatology’s Guidelines/Outcomes Committee recommends complete removal of infected hair; however, patients may desire hair-preserving treatments.¹¹ Kiken et al¹ reported success with the combination of an oral azole antifungal agent for 3 weeks to 1 month and an antifungal shampoo for 2 to 3 months. The authors proposed that oral medication eliminates scalp carriage while antifungal shampoo eliminates hair shaft concretions.¹
 

The Diagnosis: White Piedra

A fungal culture demonstrated a filamentous fungus that was identified as Trichosporon inkin via DNA sequencing, which confirmed the diagnosis of white piedra (WP).

Piedra refers to a group of fungal infections presenting as gritty nodules adherent to the hair shaft.¹ It is further categorized into black piedra, which occurs more commonly in tropical climates and is caused by Piedraia hortae, and WP, which occurs in tropical and temperate climates and is caused by the Trichosporon genus.^1-3 Among the Trichosporon genus, clinical manifestations have varied based on species; for example, T inkin commonly causes genital WP, Trichosporon ovoides commonly causes scalp WP, and Trichosporon asahii and Trichosporon mucoides have been described to cause systemic fungal infections in immunocompromised hosts.^1,4 Scalp WP most commonly occurs in children and young adults, and females are at greater risk than males.^1,2,5,6

Clinically, WP presents with pale irregular nodules along the hair shaft that are not fluorescent on Wood lamp examination.^1,6,7 Nodules are soft and easily detached from the hair shaft, unlike the hard, tightly adherent nodules seen in black piedra.^1,7 White piedra affects hair in a variety of areas including the scalp, beard, eyebrows, eyelashes, axillae, and genitals.^1,7 Affected hair may become brittle and break at points of invasion.1 Alternatively, WP may resemble tinea capitis with scalp hyperkeratosis and alopecia, though tinea typically affects the base of the hair shaft.¹ Immunocompromised patients can develop disseminated WP, and cases of progressive pneumonia, lung abscess, peritonitis, vascular access infection, and endocarditis have been reported.²

Diagnosis of WP is made through a combination of clinical findings and culture of infected hair. Potassium hydroxide preparation demonstrates sleevelike concretions formed of masses of septate hyphae with dense zones of arthrospores and blastospores.^1,2 Culture on Sabouraud agar demonstrates creamy colonies that develop a dull, gray, wrinkled surface.^1,2 Differential diagnosis includes pediculosis; however, the concretions of WP are circumferential around the hair shaft on microscopy.¹ Notably, a case of concomitant WP and pediculosis has been reported.⁸ In cases of potential pediculosis resistant to therapy, consider hair casts, which are asymptomatic, white, cylindrical concretions that encircle the hair without adherence and can therefore be differentiated from pediculosis via dermoscopy.⁹ Because this phenomenon is more commonly observed in preadolescent girls, it is hypothesized that scalp inflammation due to traction from hairstyles or atopic dermatitis contributes to the development of hair casts.^9,10 Thus, when a potassium hydroxide mount is equivocal for nits and dermoscopy demonstrates concretions that completely encircle the hair shaft, it is important to perform a microbiologic culture to rule out piedra of the hair or scalp. Other differential diagnoses include tinea capitis, black piedra, trichobacteriosis, and hair shaft abnormalities.

Transmission of WP is thought to result from a combination of poor hygiene; humidity due to climate; personal care practices such as habitually tying wet hair, applying hair oils and conditioners, or covering hair according to social customs; and close contact with an infected individual.^1,3,6 Long scalp hair potentially correlates with increased risk.^1,6 Finally, WP has been described in animals and has been isolated from soil, vegetable matter, and water.^3,10

Treatment of WP generally involves removal of infected hair, antifungal agents, and improved hygienic habits to avoid relapses. The American Academy of Dermatology’s Guidelines/Outcomes Committee recommends complete removal of infected hair; however, patients may desire hair-preserving treatments.¹¹ Kiken et al¹ reported success with the combination of an oral azole antifungal agent for 3 weeks to 1 month and an antifungal shampoo for 2 to 3 months. The authors proposed that oral medication eliminates scalp carriage while antifungal shampoo eliminates hair shaft concretions.¹
 

En coup de sabre (ECDS) is a rare subtype of linear scleroderma that is limited to the hemiface in a unilateral distribution. The lesional skin first exhibits contraction and stiffness that lead to characteristic fibrotic plaques with associated linear alopecia.¹ The pansclerotic plaques are ivory in color with hyperpigmented to violaceous borders extending as a paramedian band on the frontoparietal scalp.^2,3 The skin lesions bear resemblance to the stroke of the sabre sword, giving the condition its unique name. Many patients initially present with concerns of frontal scalp alopecia.³ Linear morphea, including the ECDS subtype, is predominantly seen in children and women, usually presenting within the first 2 decades of life.^1,4

The differential diagnoses of ECDS include focal dermal hypoplasia, steroid atrophy, localized morphea, and lupus profundus.⁵ En coup de sabre should be distinguished from progressive hemifacial atrophy (PHA)(also known as Parry-Romberg syndrome).⁶ Progressive hemifacial atrophy presents as unilateral atrophy of the face involving skin, subcutaneous tissue, muscle, and underlying bone in the distribution of the trigeminal nerve.¹ Both PHA and ECDS exist on a spectrum of linear scleroderma and may coexist in the same patient.⁶

There is a strong association with extracutaneous neurologic involvement, including seizures, ocular abnormalities, trigeminal neuralgia, and headache.^7-10 One study examining ECDS and PHA demonstrated that 44% (19/43) of patients who underwent central nervous system imaging had abnormal findings.¹¹ The majority of patients had magnetic resonance imaging with or without contrast, computed tomography, or both. The most common findings on T2-weighted images were white matter hyperintensities, mostly in subcortical and periventricular regions. The findings were bilateral in 61% (11/18) of patients and ipsilateral to the lesion in 33% (6/18) of patients.¹¹ We present a case of ECDS masquerading as alopecia in a 77-year-old woman.

Case Report

A 77-year-old white woman presented with a chief concern of hair loss on the scalp that had been present since 12 years of age. During her adult life, the scalp lesion remained unchanged with no associated symptoms. Her medical history was remarkable for hypertension and non–insulin-dependent diabetes mellitus. The patient denied any history of seizure disorders, facial paralysis, or neurologic deficits. Physical examination revealed a 13.6-cm linear, alopecic, hyperpigmented plaque extending from the left forehead and temporal scalp to the posterior occipital scalp with notable loss of underlying subcutaneous tissue (Figure). The left temporal scalp and forehead demonstrated pronounced atrophy with overlying telangiectases. Minimal depression was observed in the temporal scalp and forehead. The left cranial bone was easily palpable with no underlying subcutaneous tissue present. Computed tomography of the brain revealed no underlying skull or soft tissue abnormalities. No treatment was initiated, as the lesion was of minimal concern to the patient.

Comment

Etiology and Presentation                                                                                                                                                                                                                                      
En coup de sabre is a rare subtype of linear morphea that involves the frontoparietal scalp and forehead.^7,12,13 It manifests as a solitary, linear, fibrous plaque that involves the skin, underlying muscle, and bone.⁷ Although most cases present with a single lesion, multiple lesions can occur.⁸ The exact etiology of this disease remains to be determined but is characterized by thickening and hardening of the skin secondary to increased collagen production.⁷ The incidence of linear morphea ranges from 0.4 to 2.7 cases per 100,000 individuals and is more prevalent in white patients and women.¹⁴ Linear morphea is commonly found in children. Children are more likely to have linear morphea on the face, which can lead to lifelong disfigurement.² Although the disease peaks in the fourth decade of life for adults, most pediatric cases are diagnosed between 2 and 14 years of age.^14-16

Pathogenesis                                                                                                                                                                                                                                                       
Clinical and histopathological data suggest that a complex interaction among the vasculature, extracellular matrix, and immune system plays a role in the pathogenesis of the disease. Similar to scleroderma, the CD4 helper T cell may be involved in the fibrotic changes that occur within these lesions.¹⁷ Early in the disease process, T_H1 and T_H17 inflammatory pathways predominate. The late fibrotic changes seen in scleroderma are more associated with a shift to the T_H2 inflammatory pathway.¹⁷ Infection with Borrelia burgdorferi has been implicated abroad, but a large-scale study confirming Borrelia as a pathologic factor within morphea lesions has not been completed to date.^18-20 Some authors believe early lesions of ECDS mimic erythema chronica migrans, with the late lesions resembling acrodermatitis chronica atrophicans.²⁰

Histopathology                                                                                                                                                                                                                                     
Histopathologic findings of morphea tend to vary depending on the stage of the disease. The 2 stages of morphea can be differentiated by the degree of inflammation present histologically.^14,21 The early phase of morphea primarily affects the connective and subcutaneous tissue surrounding eccrine sweat glands.^14,21 A dense dermal and subcutaneous perivascular lymphocytic infiltrate with a mixture of lymphocytes, plasma cells, and histiocytes is commonly observed.⁵ Later stages of the disease demonstrate densely packed homogenous collagen with minimal inflammation and loss of eccrine glands and blood vessels.^14,21 The adipose tissue is generally replaced by sclerotic collagen, giving the biopsy a squared-off appearance.^5,14

Management                                                                                                                                                                                                                                                            
En coup de sabre presents a treatment challenge. In active lesions, topical or intralesional corticosteroids are considered treatment of choice.⁵ Methotrexate has proven useful in the treatment of acute and deep forms of linear morphea. A study examining methotrexate in juvenile localized scleroderma, with the majority of patients having the linear subtype, revealed that methotrexate is both efficacious and well tolerated.²² Other reports in the literature reveal efficacy with the use of intravenous corticosteroids and methotrexate combination therapy for treatment of morphea.^23,24 A longitudinal prospective study examining the use of high-dose methotrexate and oral corticosteroids for the treatment of localized scleroderma yielded positive results, with patients showing clinical improvement within 2 months of initiation of combination therapy.²⁵ Other treatments include excimer laser; calcipotriene and tacrolimus; and surgical approaches such as autologous fat grafting, grafting with muscle flaps, and tissue inserts.^21,26-31 In addition, patients can choose to forego therapy, as was the case with our patient.

Conclusion

En coup de sabre is a rare subtype of linear scleroderma that is limited to the ipsilateral scalp and face predominately in children and women. Neurologic involvement is common and should prompt a comprehensive neurologic workup in patients suspected to have ECDS or PHA. Current treatment recommendations include topical, intralesional, and oral corticosteroids; methotrexate; and surgical grafts. Although ECDS is a rare entity, more intensive research is needed on the exact pathophysiology and effective treatment options that focus on improving the cosmetic outcome in these patients. Cosmesis is the primary concern in patients with ECDS and should be managed early and appropriately to prevent long-term psychological sequelae.

En coup de sabre (ECDS) is a rare subtype of linear scleroderma that is limited to the hemiface in a unilateral distribution. The lesional skin first exhibits contraction and stiffness that lead to characteristic fibrotic plaques with associated linear alopecia.¹ The pansclerotic plaques are ivory in color with hyperpigmented to violaceous borders extending as a paramedian band on the frontoparietal scalp.^2,3 The skin lesions bear resemblance to the stroke of the sabre sword, giving the condition its unique name. Many patients initially present with concerns of frontal scalp alopecia.³ Linear morphea, including the ECDS subtype, is predominantly seen in children and women, usually presenting within the first 2 decades of life.^1,4

The differential diagnoses of ECDS include focal dermal hypoplasia, steroid atrophy, localized morphea, and lupus profundus.⁵ En coup de sabre should be distinguished from progressive hemifacial atrophy (PHA)(also known as Parry-Romberg syndrome).⁶ Progressive hemifacial atrophy presents as unilateral atrophy of the face involving skin, subcutaneous tissue, muscle, and underlying bone in the distribution of the trigeminal nerve.¹ Both PHA and ECDS exist on a spectrum of linear scleroderma and may coexist in the same patient.⁶

There is a strong association with extracutaneous neurologic involvement, including seizures, ocular abnormalities, trigeminal neuralgia, and headache.^7-10 One study examining ECDS and PHA demonstrated that 44% (19/43) of patients who underwent central nervous system imaging had abnormal findings.¹¹ The majority of patients had magnetic resonance imaging with or without contrast, computed tomography, or both. The most common findings on T2-weighted images were white matter hyperintensities, mostly in subcortical and periventricular regions. The findings were bilateral in 61% (11/18) of patients and ipsilateral to the lesion in 33% (6/18) of patients.¹¹ We present a case of ECDS masquerading as alopecia in a 77-year-old woman.

Case Report

A 77-year-old white woman presented with a chief concern of hair loss on the scalp that had been present since 12 years of age. During her adult life, the scalp lesion remained unchanged with no associated symptoms. Her medical history was remarkable for hypertension and non–insulin-dependent diabetes mellitus. The patient denied any history of seizure disorders, facial paralysis, or neurologic deficits. Physical examination revealed a 13.6-cm linear, alopecic, hyperpigmented plaque extending from the left forehead and temporal scalp to the posterior occipital scalp with notable loss of underlying subcutaneous tissue (Figure). The left temporal scalp and forehead demonstrated pronounced atrophy with overlying telangiectases. Minimal depression was observed in the temporal scalp and forehead. The left cranial bone was easily palpable with no underlying subcutaneous tissue present. Computed tomography of the brain revealed no underlying skull or soft tissue abnormalities. No treatment was initiated, as the lesion was of minimal concern to the patient.

Comment

Etiology and Presentation                                                                                                                                                                                                                                      
En coup de sabre is a rare subtype of linear morphea that involves the frontoparietal scalp and forehead.^7,12,13 It manifests as a solitary, linear, fibrous plaque that involves the skin, underlying muscle, and bone.⁷ Although most cases present with a single lesion, multiple lesions can occur.⁸ The exact etiology of this disease remains to be determined but is characterized by thickening and hardening of the skin secondary to increased collagen production.⁷ The incidence of linear morphea ranges from 0.4 to 2.7 cases per 100,000 individuals and is more prevalent in white patients and women.¹⁴ Linear morphea is commonly found in children. Children are more likely to have linear morphea on the face, which can lead to lifelong disfigurement.² Although the disease peaks in the fourth decade of life for adults, most pediatric cases are diagnosed between 2 and 14 years of age.^14-16

Pathogenesis                                                                                                                                                                                                                                                       
Clinical and histopathological data suggest that a complex interaction among the vasculature, extracellular matrix, and immune system plays a role in the pathogenesis of the disease. Similar to scleroderma, the CD4 helper T cell may be involved in the fibrotic changes that occur within these lesions.¹⁷ Early in the disease process, T_H1 and T_H17 inflammatory pathways predominate. The late fibrotic changes seen in scleroderma are more associated with a shift to the T_H2 inflammatory pathway.¹⁷ Infection with Borrelia burgdorferi has been implicated abroad, but a large-scale study confirming Borrelia as a pathologic factor within morphea lesions has not been completed to date.^18-20 Some authors believe early lesions of ECDS mimic erythema chronica migrans, with the late lesions resembling acrodermatitis chronica atrophicans.²⁰

Histopathology                                                                                                                                                                                                                                     
Histopathologic findings of morphea tend to vary depending on the stage of the disease. The 2 stages of morphea can be differentiated by the degree of inflammation present histologically.^14,21 The early phase of morphea primarily affects the connective and subcutaneous tissue surrounding eccrine sweat glands.^14,21 A dense dermal and subcutaneous perivascular lymphocytic infiltrate with a mixture of lymphocytes, plasma cells, and histiocytes is commonly observed.⁵ Later stages of the disease demonstrate densely packed homogenous collagen with minimal inflammation and loss of eccrine glands and blood vessels.^14,21 The adipose tissue is generally replaced by sclerotic collagen, giving the biopsy a squared-off appearance.^5,14

Management                                                                                                                                                                                                                                                            
En coup de sabre presents a treatment challenge. In active lesions, topical or intralesional corticosteroids are considered treatment of choice.⁵ Methotrexate has proven useful in the treatment of acute and deep forms of linear morphea. A study examining methotrexate in juvenile localized scleroderma, with the majority of patients having the linear subtype, revealed that methotrexate is both efficacious and well tolerated.²² Other reports in the literature reveal efficacy with the use of intravenous corticosteroids and methotrexate combination therapy for treatment of morphea.^23,24 A longitudinal prospective study examining the use of high-dose methotrexate and oral corticosteroids for the treatment of localized scleroderma yielded positive results, with patients showing clinical improvement within 2 months of initiation of combination therapy.²⁵ Other treatments include excimer laser; calcipotriene and tacrolimus; and surgical approaches such as autologous fat grafting, grafting with muscle flaps, and tissue inserts.^21,26-31 In addition, patients can choose to forego therapy, as was the case with our patient.

Conclusion

En coup de sabre is a rare subtype of linear scleroderma that is limited to the ipsilateral scalp and face predominately in children and women. Neurologic involvement is common and should prompt a comprehensive neurologic workup in patients suspected to have ECDS or PHA. Current treatment recommendations include topical, intralesional, and oral corticosteroids; methotrexate; and surgical grafts. Although ECDS is a rare entity, more intensive research is needed on the exact pathophysiology and effective treatment options that focus on improving the cosmetic outcome in these patients. Cosmesis is the primary concern in patients with ECDS and should be managed early and appropriately to prevent long-term psychological sequelae.

En coup de sabre (ECDS) is a rare subtype of linear scleroderma that is limited to the hemiface in a unilateral distribution. The lesional skin first exhibits contraction and stiffness that lead to characteristic fibrotic plaques with associated linear alopecia.¹ The pansclerotic plaques are ivory in color with hyperpigmented to violaceous borders extending as a paramedian band on the frontoparietal scalp.^2,3 The skin lesions bear resemblance to the stroke of the sabre sword, giving the condition its unique name. Many patients initially present with concerns of frontal scalp alopecia.³ Linear morphea, including the ECDS subtype, is predominantly seen in children and women, usually presenting within the first 2 decades of life.^1,4

The differential diagnoses of ECDS include focal dermal hypoplasia, steroid atrophy, localized morphea, and lupus profundus.⁵ En coup de sabre should be distinguished from progressive hemifacial atrophy (PHA)(also known as Parry-Romberg syndrome).⁶ Progressive hemifacial atrophy presents as unilateral atrophy of the face involving skin, subcutaneous tissue, muscle, and underlying bone in the distribution of the trigeminal nerve.¹ Both PHA and ECDS exist on a spectrum of linear scleroderma and may coexist in the same patient.⁶

There is a strong association with extracutaneous neurologic involvement, including seizures, ocular abnormalities, trigeminal neuralgia, and headache.^7-10 One study examining ECDS and PHA demonstrated that 44% (19/43) of patients who underwent central nervous system imaging had abnormal findings.¹¹ The majority of patients had magnetic resonance imaging with or without contrast, computed tomography, or both. The most common findings on T2-weighted images were white matter hyperintensities, mostly in subcortical and periventricular regions. The findings were bilateral in 61% (11/18) of patients and ipsilateral to the lesion in 33% (6/18) of patients.¹¹ We present a case of ECDS masquerading as alopecia in a 77-year-old woman.

Case Report

A 77-year-old white woman presented with a chief concern of hair loss on the scalp that had been present since 12 years of age. During her adult life, the scalp lesion remained unchanged with no associated symptoms. Her medical history was remarkable for hypertension and non–insulin-dependent diabetes mellitus. The patient denied any history of seizure disorders, facial paralysis, or neurologic deficits. Physical examination revealed a 13.6-cm linear, alopecic, hyperpigmented plaque extending from the left forehead and temporal scalp to the posterior occipital scalp with notable loss of underlying subcutaneous tissue (Figure). The left temporal scalp and forehead demonstrated pronounced atrophy with overlying telangiectases. Minimal depression was observed in the temporal scalp and forehead. The left cranial bone was easily palpable with no underlying subcutaneous tissue present. Computed tomography of the brain revealed no underlying skull or soft tissue abnormalities. No treatment was initiated, as the lesion was of minimal concern to the patient.

Comment

Etiology and Presentation                                                                                                                                                                                                                                      
En coup de sabre is a rare subtype of linear morphea that involves the frontoparietal scalp and forehead.^7,12,13 It manifests as a solitary, linear, fibrous plaque that involves the skin, underlying muscle, and bone.⁷ Although most cases present with a single lesion, multiple lesions can occur.⁸ The exact etiology of this disease remains to be determined but is characterized by thickening and hardening of the skin secondary to increased collagen production.⁷ The incidence of linear morphea ranges from 0.4 to 2.7 cases per 100,000 individuals and is more prevalent in white patients and women.¹⁴ Linear morphea is commonly found in children. Children are more likely to have linear morphea on the face, which can lead to lifelong disfigurement.² Although the disease peaks in the fourth decade of life for adults, most pediatric cases are diagnosed between 2 and 14 years of age.^14-16

Pathogenesis                                                                                                                                                                                                                                                       
Clinical and histopathological data suggest that a complex interaction among the vasculature, extracellular matrix, and immune system plays a role in the pathogenesis of the disease. Similar to scleroderma, the CD4 helper T cell may be involved in the fibrotic changes that occur within these lesions.¹⁷ Early in the disease process, T_H1 and T_H17 inflammatory pathways predominate. The late fibrotic changes seen in scleroderma are more associated with a shift to the T_H2 inflammatory pathway.¹⁷ Infection with Borrelia burgdorferi has been implicated abroad, but a large-scale study confirming Borrelia as a pathologic factor within morphea lesions has not been completed to date.^18-20 Some authors believe early lesions of ECDS mimic erythema chronica migrans, with the late lesions resembling acrodermatitis chronica atrophicans.²⁰

Histopathology                                                                                                                                                                                                                                     
Histopathologic findings of morphea tend to vary depending on the stage of the disease. The 2 stages of morphea can be differentiated by the degree of inflammation present histologically.^14,21 The early phase of morphea primarily affects the connective and subcutaneous tissue surrounding eccrine sweat glands.^14,21 A dense dermal and subcutaneous perivascular lymphocytic infiltrate with a mixture of lymphocytes, plasma cells, and histiocytes is commonly observed.⁵ Later stages of the disease demonstrate densely packed homogenous collagen with minimal inflammation and loss of eccrine glands and blood vessels.^14,21 The adipose tissue is generally replaced by sclerotic collagen, giving the biopsy a squared-off appearance.^5,14

Management                                                                                                                                                                                                                                                            
En coup de sabre presents a treatment challenge. In active lesions, topical or intralesional corticosteroids are considered treatment of choice.⁵ Methotrexate has proven useful in the treatment of acute and deep forms of linear morphea. A study examining methotrexate in juvenile localized scleroderma, with the majority of patients having the linear subtype, revealed that methotrexate is both efficacious and well tolerated.²² Other reports in the literature reveal efficacy with the use of intravenous corticosteroids and methotrexate combination therapy for treatment of morphea.^23,24 A longitudinal prospective study examining the use of high-dose methotrexate and oral corticosteroids for the treatment of localized scleroderma yielded positive results, with patients showing clinical improvement within 2 months of initiation of combination therapy.²⁵ Other treatments include excimer laser; calcipotriene and tacrolimus; and surgical approaches such as autologous fat grafting, grafting with muscle flaps, and tissue inserts.^21,26-31 In addition, patients can choose to forego therapy, as was the case with our patient.

Conclusion

En coup de sabre is a rare subtype of linear scleroderma that is limited to the ipsilateral scalp and face predominately in children and women. Neurologic involvement is common and should prompt a comprehensive neurologic workup in patients suspected to have ECDS or PHA. Current treatment recommendations include topical, intralesional, and oral corticosteroids; methotrexate; and surgical grafts. Although ECDS is a rare entity, more intensive research is needed on the exact pathophysiology and effective treatment options that focus on improving the cosmetic outcome in these patients. Cosmesis is the primary concern in patients with ECDS and should be managed early and appropriately to prevent long-term psychological sequelae.

Pityriasis amiantacea (PA) is characterized by adherence of hair shafts proximally.¹ It has been associated with dermatologic conditions and rarely with medications. We describe a woman who developed PA following a bone marrow transplant with melphalan conditioning. We also review drug-induced PA and disorders that have been linked to this condition.

Case Report

A 67-year-old woman with a history of multiple myeloma was treated with 7 courses of chemotherapy (cyclophosphamide, bortezomib, prednisone). One month later, the patient underwent a bone marrow transplant with melphalan conditioning due to residual plasma cell myeloma. Following the transplant, she developed complete scalp alopecia. Prior to and following transplant, the patient’s hair care regimen included washing her hair and scalp every other day with over-the-counter “natural” shampoos. During drug-induced alopecia, the hair washing became less frequent.

The patient left the hospital 4 weeks posttransplant; her hair had started to regrow, but its appearance was altered. Posttransplant, the patient was maintained on bortezomib every other week and zoledronate once per month. She continued to develop multiple lesions in the scalp hairs during the following 4 months.

Eight months posttransplant she presented for evaluation of the scalp hair. Clinical examination showed hairs that were entwined together proximally, resulting in matting of the hair (Figure 1). A diagnosis of PA was established based on the clinical examination.

Treatment included mineral oil application to the scalp under occlusion each evening, followed by morning washing with coal tar 0.5%, salicylic acid 6%, or ketoconazole 2% shampoo in a repeating sequential manner. Within 1 month there was complete resolution of the scalp condition (Figure 2).

Comment

Clinical Presentation                                                                                                                                                                                                                                                                                                                                                             
Pityriasis amiantacea is characterized by thick excessive scale of the scalp¹; it was initially described by Alibert² in 1832. He described the gross appearance of the scales as resembling the feathers of young birds, which naturalists dub “amiante” or asbestoslike.^1,2 In 1917, Gougerot³ explored infectious etiologies of this condition by describing cases of impetigo that transitioned into PA.¹ Later, in 1929, Photinos⁴ described fungal origins of PA, giving credence to “tinea amiantacea.”¹ However, more recent analyses failed to isolate fungus.^5-7 As such, pityriasis (scaling) amiantacea is the more appropriate term, as emphasized by Brown⁸ in 1948. The cause of PA remains unclear; it is hypothesized that the condition is a reaction to underlying inflammatory dermatoses, though concurrent bacterial or fungal infection may be present.^5,9

Prevalence                                                                                                                                                                                                                                                                                                                                                                             
Pityriasis amiantacea is considered to be most prevalent in pediatric patients and young adults; it is more common in females.^1,9,10 In a review of 85 PA patients, more than 80% were women (n=69), and the mean age at presentation was 23.8 years. Approximately half of these patients had widespread scalp lesions (n=42); however, focal localized lesions were common.⁹ No hereditary patterns have been described, though 3 pairs of the 10 patients with PA in Ring and Kaplan’s⁷ review were siblings.

Histopathology                                                                                                                                                                                                                                                                                                                                                                 
Submission of hair specimens to histopathology usually is not performed since the diagnosis often is established based on the clinical presentation.⁵ However, submitted specimens have demonstrated spongiosis and parakeratosis along with reduction in the size of the sebaceous glands.^1,9 Additionally, follicular keratosis that surrounds the hair shafts with a sheath of horn is present.⁹ Acanthosis and migration of lymphocytes into the epidermis also have been found.¹ Often, Staphylococcus aureus isolates are detected.^9,13
 

Differential Diagnosis                                                                                                                                                                                                                                                                                                                                                                   
The clinical differential diagnosis of PA includes hair casts,¹¹ pediculosis,¹⁴ and tinea capitis.¹² In PA, thick scales surround hair shafts and thus bind down tufts of hair.⁹ In patients with pediculosis, nits are attached to the hair shaft at an angle and do not entirely envelop the hair shaft.¹⁴ In addition, PA may be complicated by impetiginization; bacteria often are found in the keratin surrounding the hair shaft and represent either normal flora or secondary infection.^1,15 It has been speculated that microbial biofilms from S aureus and Staphylococcus epidermidis promote agglomeration of hair shafts and adherent scale.¹⁶ Bona fide dermatophyte infection of the scalp also may be concurrently present.¹²

Treatment                                                                                                                                                                                                                                                                                                                                                                                     
Our treatment included occlusion with mineral oil to loosen the scales from the scalp in tandem with shampoos traditionally used in patients with seborrheic dermatitis or psoriasis. Timely treatment is important to prevent scarring alopecia.^13,17 Pityriasis amiantacea may be treatment resistant, and there are no specific therapeutic guidelines; rather, therapy should be targeted at the suspected underlying condition.¹⁷ Treatment generally includes keratolytic agents, such as salicylic acid.¹⁸ These agents allow enhanced penetration of other topical agents.¹⁹ Topical antifungal shampoos such as ketoconazole and ciclopirox are recommended,¹⁸ though other topical agents, such as coal tar and zinc pyrithione, also may benefit patients.¹³ Topical corticosteroids may be used if the condition is linked with psoriasis.¹³ Systemic antibiotics are added if S aureus superinfection is suspected.⁹

A single report described successful management of a patient with severe refractory PA who was treated with the tumor necrosis factor (TNF) α inhibitor infliximab.¹³ A 47-year-old woman presented with thick adherent scale on the scalp. She was treated with coal tar for 18 months but showed no improvement; the patient was subsequently prescribed salicylic acid 10%, clobetasol solution, and coal tar shampoo. After 3 months, when no improvement was observed, the patient was offered infliximab but declined. For 6 years the patient was treated with salicylic acid 20%, clobetasol (foam, lotion, shampoo, and solution), and coal tar shampoo without improvement. She then consented to infliximab therapy; after 3 infusions at weeks 0, 2, and 6, she demonstrated notable improvement. The patient was maintained on infliximab every 8 weeks.¹³

Pathogenesis                                                                                                                                                                                                                                                                                                                                                                                 
The pathogenesis of PA has yet to be definitively established, and the condition is usually idiopathic. In addition to bacterial or fungal etiologies,^3,4 PA has been linked to medications (Table 1)^16,20,21 and systemic conditions (Table 2).^{1,3,5,7-10,12,22-25}

A PubMed search of articles indexed for MEDLINE using the search terms amiantacea, bone, drug, hair marrow, malignancy, melphalan, pityriasis, tinea, and transplant yielded 4 patients—2 men and 2 women (including our patient)—with possible drug-induced PA (Table 1)^16,20,21; however, the onset after 2 years of medication (TNF-α inhibitors) or resolution while still receiving the agent (vemurafenib) makes the drug-induced linkage weak. The patients ranged in age from 21 to 67 years, with the median age being 37.5 years. Medications included melphalan, TNF-α inhibitors (adalimumab, infliximab),^16,21 and vemurafenib²⁰; it is interesting that infliximab was the medication associated with eliciting PA in 1 patient yet was an effective therapy in another patient with treatment-resistant PA. The onset of PA occurred between 1 month (melphalan) and 24 months (TNF-α inhibitors) after drug initiation. The patients’ associated diseases included Crohn disease,^16,21 metastatic melanoma,²⁰ and multiple myeloma.

Other conditions have been described in patients with PA (Table 2). Indeed, PA may be a manifestation of an underlying inflammatory skin disease.⁹ In addition to dermatologic conditions, procedures or malignancy may be associated with the disease, as demonstrated in our patient. Most commonly, PA is seen in association with psoriasis and seborrheic dermatitis; atopic dermatitis, bacterial infection, fungal infection, lichen planus, and neurodermatitis also have been associated with PA.^{1,3,5,7-10,12,18,22-25}

Conclusion

Pityriasis amiantacea is a benign condition affecting the scalp hair. Albeit uncommon, it may appear in patients treated with medications such as melphalan, TNF-α inhibitors, and vemurafenib. In addition, it has been described in individuals with dermatologic conditions, systemic procedures, or underlying malignancy. Our patient developed PA following a bone marrow transplant after receiving conditioning with melphalan.

Pityriasis amiantacea (PA) is characterized by adherence of hair shafts proximally.¹ It has been associated with dermatologic conditions and rarely with medications. We describe a woman who developed PA following a bone marrow transplant with melphalan conditioning. We also review drug-induced PA and disorders that have been linked to this condition.

Case Report

A 67-year-old woman with a history of multiple myeloma was treated with 7 courses of chemotherapy (cyclophosphamide, bortezomib, prednisone). One month later, the patient underwent a bone marrow transplant with melphalan conditioning due to residual plasma cell myeloma. Following the transplant, she developed complete scalp alopecia. Prior to and following transplant, the patient’s hair care regimen included washing her hair and scalp every other day with over-the-counter “natural” shampoos. During drug-induced alopecia, the hair washing became less frequent.

The patient left the hospital 4 weeks posttransplant; her hair had started to regrow, but its appearance was altered. Posttransplant, the patient was maintained on bortezomib every other week and zoledronate once per month. She continued to develop multiple lesions in the scalp hairs during the following 4 months.

Eight months posttransplant she presented for evaluation of the scalp hair. Clinical examination showed hairs that were entwined together proximally, resulting in matting of the hair (Figure 1). A diagnosis of PA was established based on the clinical examination.

Treatment included mineral oil application to the scalp under occlusion each evening, followed by morning washing with coal tar 0.5%, salicylic acid 6%, or ketoconazole 2% shampoo in a repeating sequential manner. Within 1 month there was complete resolution of the scalp condition (Figure 2).

Comment

Clinical Presentation                                                                                                                                                                                                                                                                                                                                                             
Pityriasis amiantacea is characterized by thick excessive scale of the scalp¹; it was initially described by Alibert² in 1832. He described the gross appearance of the scales as resembling the feathers of young birds, which naturalists dub “amiante” or asbestoslike.^1,2 In 1917, Gougerot³ explored infectious etiologies of this condition by describing cases of impetigo that transitioned into PA.¹ Later, in 1929, Photinos⁴ described fungal origins of PA, giving credence to “tinea amiantacea.”¹ However, more recent analyses failed to isolate fungus.^5-7 As such, pityriasis (scaling) amiantacea is the more appropriate term, as emphasized by Brown⁸ in 1948. The cause of PA remains unclear; it is hypothesized that the condition is a reaction to underlying inflammatory dermatoses, though concurrent bacterial or fungal infection may be present.^5,9

Prevalence                                                                                                                                                                                                                                                                                                                                                                             
Pityriasis amiantacea is considered to be most prevalent in pediatric patients and young adults; it is more common in females.^1,9,10 In a review of 85 PA patients, more than 80% were women (n=69), and the mean age at presentation was 23.8 years. Approximately half of these patients had widespread scalp lesions (n=42); however, focal localized lesions were common.⁹ No hereditary patterns have been described, though 3 pairs of the 10 patients with PA in Ring and Kaplan’s⁷ review were siblings.

Histopathology                                                                                                                                                                                                                                                                                                                                                                 
Submission of hair specimens to histopathology usually is not performed since the diagnosis often is established based on the clinical presentation.⁵ However, submitted specimens have demonstrated spongiosis and parakeratosis along with reduction in the size of the sebaceous glands.^1,9 Additionally, follicular keratosis that surrounds the hair shafts with a sheath of horn is present.⁹ Acanthosis and migration of lymphocytes into the epidermis also have been found.¹ Often, Staphylococcus aureus isolates are detected.^9,13
 

Differential Diagnosis                                                                                                                                                                                                                                                                                                                                                                   
The clinical differential diagnosis of PA includes hair casts,¹¹ pediculosis,¹⁴ and tinea capitis.¹² In PA, thick scales surround hair shafts and thus bind down tufts of hair.⁹ In patients with pediculosis, nits are attached to the hair shaft at an angle and do not entirely envelop the hair shaft.¹⁴ In addition, PA may be complicated by impetiginization; bacteria often are found in the keratin surrounding the hair shaft and represent either normal flora or secondary infection.^1,15 It has been speculated that microbial biofilms from S aureus and Staphylococcus epidermidis promote agglomeration of hair shafts and adherent scale.¹⁶ Bona fide dermatophyte infection of the scalp also may be concurrently present.¹²

Treatment                                                                                                                                                                                                                                                                                                                                                                                     
Our treatment included occlusion with mineral oil to loosen the scales from the scalp in tandem with shampoos traditionally used in patients with seborrheic dermatitis or psoriasis. Timely treatment is important to prevent scarring alopecia.^13,17 Pityriasis amiantacea may be treatment resistant, and there are no specific therapeutic guidelines; rather, therapy should be targeted at the suspected underlying condition.¹⁷ Treatment generally includes keratolytic agents, such as salicylic acid.¹⁸ These agents allow enhanced penetration of other topical agents.¹⁹ Topical antifungal shampoos such as ketoconazole and ciclopirox are recommended,¹⁸ though other topical agents, such as coal tar and zinc pyrithione, also may benefit patients.¹³ Topical corticosteroids may be used if the condition is linked with psoriasis.¹³ Systemic antibiotics are added if S aureus superinfection is suspected.⁹

A single report described successful management of a patient with severe refractory PA who was treated with the tumor necrosis factor (TNF) α inhibitor infliximab.¹³ A 47-year-old woman presented with thick adherent scale on the scalp. She was treated with coal tar for 18 months but showed no improvement; the patient was subsequently prescribed salicylic acid 10%, clobetasol solution, and coal tar shampoo. After 3 months, when no improvement was observed, the patient was offered infliximab but declined. For 6 years the patient was treated with salicylic acid 20%, clobetasol (foam, lotion, shampoo, and solution), and coal tar shampoo without improvement. She then consented to infliximab therapy; after 3 infusions at weeks 0, 2, and 6, she demonstrated notable improvement. The patient was maintained on infliximab every 8 weeks.¹³

Pathogenesis                                                                                                                                                                                                                                                                                                                                                                                 
The pathogenesis of PA has yet to be definitively established, and the condition is usually idiopathic. In addition to bacterial or fungal etiologies,^3,4 PA has been linked to medications (Table 1)^16,20,21 and systemic conditions (Table 2).^{1,3,5,7-10,12,22-25}

A PubMed search of articles indexed for MEDLINE using the search terms amiantacea, bone, drug, hair marrow, malignancy, melphalan, pityriasis, tinea, and transplant yielded 4 patients—2 men and 2 women (including our patient)—with possible drug-induced PA (Table 1)^16,20,21; however, the onset after 2 years of medication (TNF-α inhibitors) or resolution while still receiving the agent (vemurafenib) makes the drug-induced linkage weak. The patients ranged in age from 21 to 67 years, with the median age being 37.5 years. Medications included melphalan, TNF-α inhibitors (adalimumab, infliximab),^16,21 and vemurafenib²⁰; it is interesting that infliximab was the medication associated with eliciting PA in 1 patient yet was an effective therapy in another patient with treatment-resistant PA. The onset of PA occurred between 1 month (melphalan) and 24 months (TNF-α inhibitors) after drug initiation. The patients’ associated diseases included Crohn disease,^16,21 metastatic melanoma,²⁰ and multiple myeloma.

Other conditions have been described in patients with PA (Table 2). Indeed, PA may be a manifestation of an underlying inflammatory skin disease.⁹ In addition to dermatologic conditions, procedures or malignancy may be associated with the disease, as demonstrated in our patient. Most commonly, PA is seen in association with psoriasis and seborrheic dermatitis; atopic dermatitis, bacterial infection, fungal infection, lichen planus, and neurodermatitis also have been associated with PA.^{1,3,5,7-10,12,18,22-25}

Conclusion

Pityriasis amiantacea is a benign condition affecting the scalp hair. Albeit uncommon, it may appear in patients treated with medications such as melphalan, TNF-α inhibitors, and vemurafenib. In addition, it has been described in individuals with dermatologic conditions, systemic procedures, or underlying malignancy. Our patient developed PA following a bone marrow transplant after receiving conditioning with melphalan.

Pityriasis amiantacea (PA) is characterized by adherence of hair shafts proximally.¹ It has been associated with dermatologic conditions and rarely with medications. We describe a woman who developed PA following a bone marrow transplant with melphalan conditioning. We also review drug-induced PA and disorders that have been linked to this condition.

Case Report

A 67-year-old woman with a history of multiple myeloma was treated with 7 courses of chemotherapy (cyclophosphamide, bortezomib, prednisone). One month later, the patient underwent a bone marrow transplant with melphalan conditioning due to residual plasma cell myeloma. Following the transplant, she developed complete scalp alopecia. Prior to and following transplant, the patient’s hair care regimen included washing her hair and scalp every other day with over-the-counter “natural” shampoos. During drug-induced alopecia, the hair washing became less frequent.

The patient left the hospital 4 weeks posttransplant; her hair had started to regrow, but its appearance was altered. Posttransplant, the patient was maintained on bortezomib every other week and zoledronate once per month. She continued to develop multiple lesions in the scalp hairs during the following 4 months.

Eight months posttransplant she presented for evaluation of the scalp hair. Clinical examination showed hairs that were entwined together proximally, resulting in matting of the hair (Figure 1). A diagnosis of PA was established based on the clinical examination.

Treatment included mineral oil application to the scalp under occlusion each evening, followed by morning washing with coal tar 0.5%, salicylic acid 6%, or ketoconazole 2% shampoo in a repeating sequential manner. Within 1 month there was complete resolution of the scalp condition (Figure 2).

Comment

Clinical Presentation                                                                                                                                                                                                                                                                                                                                                             
Pityriasis amiantacea is characterized by thick excessive scale of the scalp¹; it was initially described by Alibert² in 1832. He described the gross appearance of the scales as resembling the feathers of young birds, which naturalists dub “amiante” or asbestoslike.^1,2 In 1917, Gougerot³ explored infectious etiologies of this condition by describing cases of impetigo that transitioned into PA.¹ Later, in 1929, Photinos⁴ described fungal origins of PA, giving credence to “tinea amiantacea.”¹ However, more recent analyses failed to isolate fungus.^5-7 As such, pityriasis (scaling) amiantacea is the more appropriate term, as emphasized by Brown⁸ in 1948. The cause of PA remains unclear; it is hypothesized that the condition is a reaction to underlying inflammatory dermatoses, though concurrent bacterial or fungal infection may be present.^5,9

Prevalence                                                                                                                                                                                                                                                                                                                                                                             
Pityriasis amiantacea is considered to be most prevalent in pediatric patients and young adults; it is more common in females.^1,9,10 In a review of 85 PA patients, more than 80% were women (n=69), and the mean age at presentation was 23.8 years. Approximately half of these patients had widespread scalp lesions (n=42); however, focal localized lesions were common.⁹ No hereditary patterns have been described, though 3 pairs of the 10 patients with PA in Ring and Kaplan’s⁷ review were siblings.

Histopathology                                                                                                                                                                                                                                                                                                                                                                 
Submission of hair specimens to histopathology usually is not performed since the diagnosis often is established based on the clinical presentation.⁵ However, submitted specimens have demonstrated spongiosis and parakeratosis along with reduction in the size of the sebaceous glands.^1,9 Additionally, follicular keratosis that surrounds the hair shafts with a sheath of horn is present.⁹ Acanthosis and migration of lymphocytes into the epidermis also have been found.¹ Often, Staphylococcus aureus isolates are detected.^9,13
 

Differential Diagnosis                                                                                                                                                                                                                                                                                                                                                                   
The clinical differential diagnosis of PA includes hair casts,¹¹ pediculosis,¹⁴ and tinea capitis.¹² In PA, thick scales surround hair shafts and thus bind down tufts of hair.⁹ In patients with pediculosis, nits are attached to the hair shaft at an angle and do not entirely envelop the hair shaft.¹⁴ In addition, PA may be complicated by impetiginization; bacteria often are found in the keratin surrounding the hair shaft and represent either normal flora or secondary infection.^1,15 It has been speculated that microbial biofilms from S aureus and Staphylococcus epidermidis promote agglomeration of hair shafts and adherent scale.¹⁶ Bona fide dermatophyte infection of the scalp also may be concurrently present.¹²

Treatment                                                                                                                                                                                                                                                                                                                                                                                     
Our treatment included occlusion with mineral oil to loosen the scales from the scalp in tandem with shampoos traditionally used in patients with seborrheic dermatitis or psoriasis. Timely treatment is important to prevent scarring alopecia.^13,17 Pityriasis amiantacea may be treatment resistant, and there are no specific therapeutic guidelines; rather, therapy should be targeted at the suspected underlying condition.¹⁷ Treatment generally includes keratolytic agents, such as salicylic acid.¹⁸ These agents allow enhanced penetration of other topical agents.¹⁹ Topical antifungal shampoos such as ketoconazole and ciclopirox are recommended,¹⁸ though other topical agents, such as coal tar and zinc pyrithione, also may benefit patients.¹³ Topical corticosteroids may be used if the condition is linked with psoriasis.¹³ Systemic antibiotics are added if S aureus superinfection is suspected.⁹

A single report described successful management of a patient with severe refractory PA who was treated with the tumor necrosis factor (TNF) α inhibitor infliximab.¹³ A 47-year-old woman presented with thick adherent scale on the scalp. She was treated with coal tar for 18 months but showed no improvement; the patient was subsequently prescribed salicylic acid 10%, clobetasol solution, and coal tar shampoo. After 3 months, when no improvement was observed, the patient was offered infliximab but declined. For 6 years the patient was treated with salicylic acid 20%, clobetasol (foam, lotion, shampoo, and solution), and coal tar shampoo without improvement. She then consented to infliximab therapy; after 3 infusions at weeks 0, 2, and 6, she demonstrated notable improvement. The patient was maintained on infliximab every 8 weeks.¹³

Pathogenesis                                                                                                                                                                                                                                                                                                                                                                                 
The pathogenesis of PA has yet to be definitively established, and the condition is usually idiopathic. In addition to bacterial or fungal etiologies,^3,4 PA has been linked to medications (Table 1)^16,20,21 and systemic conditions (Table 2).^{1,3,5,7-10,12,22-25}

A PubMed search of articles indexed for MEDLINE using the search terms amiantacea, bone, drug, hair marrow, malignancy, melphalan, pityriasis, tinea, and transplant yielded 4 patients—2 men and 2 women (including our patient)—with possible drug-induced PA (Table 1)^16,20,21; however, the onset after 2 years of medication (TNF-α inhibitors) or resolution while still receiving the agent (vemurafenib) makes the drug-induced linkage weak. The patients ranged in age from 21 to 67 years, with the median age being 37.5 years. Medications included melphalan, TNF-α inhibitors (adalimumab, infliximab),^16,21 and vemurafenib²⁰; it is interesting that infliximab was the medication associated with eliciting PA in 1 patient yet was an effective therapy in another patient with treatment-resistant PA. The onset of PA occurred between 1 month (melphalan) and 24 months (TNF-α inhibitors) after drug initiation. The patients’ associated diseases included Crohn disease,^16,21 metastatic melanoma,²⁰ and multiple myeloma.

Other conditions have been described in patients with PA (Table 2). Indeed, PA may be a manifestation of an underlying inflammatory skin disease.⁹ In addition to dermatologic conditions, procedures or malignancy may be associated with the disease, as demonstrated in our patient. Most commonly, PA is seen in association with psoriasis and seborrheic dermatitis; atopic dermatitis, bacterial infection, fungal infection, lichen planus, and neurodermatitis also have been associated with PA.^{1,3,5,7-10,12,18,22-25}

Conclusion

Pityriasis amiantacea is a benign condition affecting the scalp hair. Albeit uncommon, it may appear in patients treated with medications such as melphalan, TNF-α inhibitors, and vemurafenib. In addition, it has been described in individuals with dermatologic conditions, systemic procedures, or underlying malignancy. Our patient developed PA following a bone marrow transplant after receiving conditioning with melphalan.

To the Editor:

A 2-year-old boy was referred to our pediatric dermatology clinic by his pediatrician for an enlarging mass on the mid frontal scalp. The lesion had been present since birth and slowly enlarged. His parents thought the lesion was mostly asymptomatic; however, if it was irritated, the child would cry. He was otherwise healthy and had no history of skin conditions. There was no family history of skin conditions, birthmarks, or vascular malformations. On physical examination, we observed an isolated, approximately 3-cm, well-circumscribed, mobile, flesh-colored and violaceous nodule on the mid frontal scalp (Figure 1). At that time our differential diagnosis included a complex hemangioma or other vascular proliferation, nevus lipomatosis, or even a soft-tissue malignancy such as a sarcoma. Prior to biopsy, we ordered magnetic resonance imaging (MRI) to evaluate for intracranial extension of the lesion. The MRI revealed a 3.2-cm frontal midline scalp mass with complex imaging characteristics, and the radiologist gave a differential diagnosis of hemangioma, teratoma, or less likely liposarcoma (Figure 2). Fortunately, there was no evidence of central nervous system or intracranial invasion. We then proceeded with excisional biopsy, which grossly revealed a nodular, well-circumscribed, yellow mass (Figure 3). The wound was closed with primary closure. Histologically, there was a lobulated tumor with thin, well-vascularized connective tissue septa within a myxoid stroma (Figure 4A). The tumor was composed of lipocytes in varying stages of maturity without obvious nuclear atypia (Figures 4B and 4C), leading to a diagnosis of lipoblastoma.

Lipoblastoma (also known as an embryonic lipoma) is a rare variant of lipoma. It is a benign neoplasm of immature white fat cells primarily seen in children younger than 3 years. It is reportedly twice as common in boys versus girls. Lipoblastomas present as enlarging, soft, mobile, painless nodules, usually 3 to 5 cm in diameter. The extremities are the favored location, but they also have been described on the head, neck, and trunk. Additionally, mediastinal and retroperitoneal lipoblastomas have been documented.¹ The tumors may be symptomatic, particularly when involving the neck or mediastinum. In rare instances, they may present with respiratory distress.² Multiple cases of head and neck lipoblastomas have been published in the English-language literature.³ Growing evidence supports that a chromosomal breakpoint abnormality at 8q11-q13 may be implicated in the pathogenesis.⁴

Most lipoblastomas are well circumscribed, encapsulated, and limited to the subcutis. However, lipoblastomatosis is the diffuse counterpart to lipoblastoma, affecting deeper soft tissue and often infiltrating adjacent skeletal muscle.¹

Diagnosis is made by histologic evaluation. Lipoblastoma appears as immature fat cells in varying stages of maturity with septa separating them into lobules. There should not be nuclear atypia.⁵ The histologic differential diagnosis includes other adipose tumors, most chiefly myxoid liposarcoma. These tumors have a lobular pattern without prominent septae and contain nuclear atypia with atypical mitotic figures. Myxoid liposarcoma has an infiltrating pattern similar to lipoblastomatosis and has a metastatic rate up to 60%.⁶ Imaging studies such as MRI are helpful in diagnosis, particularly in head and neck or visceral cases.³ The treatment of choice of lipoblastoma is wide excision. With complete removal, tumors rarely recur. Recurrences are more common in lipoblastomatosis or with incompletely excised primary lesions.³ A 14% to 24% recurrence rate has been recognized. Cytogenic analysis of lipomatous tumors has begun to reveal translocations in chromosome 8q11-13 region breakpoint abnormalities and translocations, specifically involving the pleomorphic adenoma gene 1, PLAG1, as the oncogenic target in lipoblastoma.⁶ Identification of these molecular mutations may provide aid in differentiating histologically similar-appearing tumors in the future.

This case illustrates a rare benign childhood tumor that can be difficult to diagnose prior to histologic examination. Our patient did not fit the typical description of a lipoblastoma, as his tumor was axially located as opposed to the more common peripheral presentation. We aim to raise awareness of this diagnosis as more cases are being recognized.

To the Editor:

A 2-year-old boy was referred to our pediatric dermatology clinic by his pediatrician for an enlarging mass on the mid frontal scalp. The lesion had been present since birth and slowly enlarged. His parents thought the lesion was mostly asymptomatic; however, if it was irritated, the child would cry. He was otherwise healthy and had no history of skin conditions. There was no family history of skin conditions, birthmarks, or vascular malformations. On physical examination, we observed an isolated, approximately 3-cm, well-circumscribed, mobile, flesh-colored and violaceous nodule on the mid frontal scalp (Figure 1). At that time our differential diagnosis included a complex hemangioma or other vascular proliferation, nevus lipomatosis, or even a soft-tissue malignancy such as a sarcoma. Prior to biopsy, we ordered magnetic resonance imaging (MRI) to evaluate for intracranial extension of the lesion. The MRI revealed a 3.2-cm frontal midline scalp mass with complex imaging characteristics, and the radiologist gave a differential diagnosis of hemangioma, teratoma, or less likely liposarcoma (Figure 2). Fortunately, there was no evidence of central nervous system or intracranial invasion. We then proceeded with excisional biopsy, which grossly revealed a nodular, well-circumscribed, yellow mass (Figure 3). The wound was closed with primary closure. Histologically, there was a lobulated tumor with thin, well-vascularized connective tissue septa within a myxoid stroma (Figure 4A). The tumor was composed of lipocytes in varying stages of maturity without obvious nuclear atypia (Figures 4B and 4C), leading to a diagnosis of lipoblastoma.

Lipoblastoma (also known as an embryonic lipoma) is a rare variant of lipoma. It is a benign neoplasm of immature white fat cells primarily seen in children younger than 3 years. It is reportedly twice as common in boys versus girls. Lipoblastomas present as enlarging, soft, mobile, painless nodules, usually 3 to 5 cm in diameter. The extremities are the favored location, but they also have been described on the head, neck, and trunk. Additionally, mediastinal and retroperitoneal lipoblastomas have been documented.¹ The tumors may be symptomatic, particularly when involving the neck or mediastinum. In rare instances, they may present with respiratory distress.² Multiple cases of head and neck lipoblastomas have been published in the English-language literature.³ Growing evidence supports that a chromosomal breakpoint abnormality at 8q11-q13 may be implicated in the pathogenesis.⁴

Most lipoblastomas are well circumscribed, encapsulated, and limited to the subcutis. However, lipoblastomatosis is the diffuse counterpart to lipoblastoma, affecting deeper soft tissue and often infiltrating adjacent skeletal muscle.¹

Diagnosis is made by histologic evaluation. Lipoblastoma appears as immature fat cells in varying stages of maturity with septa separating them into lobules. There should not be nuclear atypia.⁵ The histologic differential diagnosis includes other adipose tumors, most chiefly myxoid liposarcoma. These tumors have a lobular pattern without prominent septae and contain nuclear atypia with atypical mitotic figures. Myxoid liposarcoma has an infiltrating pattern similar to lipoblastomatosis and has a metastatic rate up to 60%.⁶ Imaging studies such as MRI are helpful in diagnosis, particularly in head and neck or visceral cases.³ The treatment of choice of lipoblastoma is wide excision. With complete removal, tumors rarely recur. Recurrences are more common in lipoblastomatosis or with incompletely excised primary lesions.³ A 14% to 24% recurrence rate has been recognized. Cytogenic analysis of lipomatous tumors has begun to reveal translocations in chromosome 8q11-13 region breakpoint abnormalities and translocations, specifically involving the pleomorphic adenoma gene 1, PLAG1, as the oncogenic target in lipoblastoma.⁶ Identification of these molecular mutations may provide aid in differentiating histologically similar-appearing tumors in the future.

This case illustrates a rare benign childhood tumor that can be difficult to diagnose prior to histologic examination. Our patient did not fit the typical description of a lipoblastoma, as his tumor was axially located as opposed to the more common peripheral presentation. We aim to raise awareness of this diagnosis as more cases are being recognized.

To the Editor:

A 2-year-old boy was referred to our pediatric dermatology clinic by his pediatrician for an enlarging mass on the mid frontal scalp. The lesion had been present since birth and slowly enlarged. His parents thought the lesion was mostly asymptomatic; however, if it was irritated, the child would cry. He was otherwise healthy and had no history of skin conditions. There was no family history of skin conditions, birthmarks, or vascular malformations. On physical examination, we observed an isolated, approximately 3-cm, well-circumscribed, mobile, flesh-colored and violaceous nodule on the mid frontal scalp (Figure 1). At that time our differential diagnosis included a complex hemangioma or other vascular proliferation, nevus lipomatosis, or even a soft-tissue malignancy such as a sarcoma. Prior to biopsy, we ordered magnetic resonance imaging (MRI) to evaluate for intracranial extension of the lesion. The MRI revealed a 3.2-cm frontal midline scalp mass with complex imaging characteristics, and the radiologist gave a differential diagnosis of hemangioma, teratoma, or less likely liposarcoma (Figure 2). Fortunately, there was no evidence of central nervous system or intracranial invasion. We then proceeded with excisional biopsy, which grossly revealed a nodular, well-circumscribed, yellow mass (Figure 3). The wound was closed with primary closure. Histologically, there was a lobulated tumor with thin, well-vascularized connective tissue septa within a myxoid stroma (Figure 4A). The tumor was composed of lipocytes in varying stages of maturity without obvious nuclear atypia (Figures 4B and 4C), leading to a diagnosis of lipoblastoma.

Lipoblastoma (also known as an embryonic lipoma) is a rare variant of lipoma. It is a benign neoplasm of immature white fat cells primarily seen in children younger than 3 years. It is reportedly twice as common in boys versus girls. Lipoblastomas present as enlarging, soft, mobile, painless nodules, usually 3 to 5 cm in diameter. The extremities are the favored location, but they also have been described on the head, neck, and trunk. Additionally, mediastinal and retroperitoneal lipoblastomas have been documented.¹ The tumors may be symptomatic, particularly when involving the neck or mediastinum. In rare instances, they may present with respiratory distress.² Multiple cases of head and neck lipoblastomas have been published in the English-language literature.³ Growing evidence supports that a chromosomal breakpoint abnormality at 8q11-q13 may be implicated in the pathogenesis.⁴

Most lipoblastomas are well circumscribed, encapsulated, and limited to the subcutis. However, lipoblastomatosis is the diffuse counterpart to lipoblastoma, affecting deeper soft tissue and often infiltrating adjacent skeletal muscle.¹

Diagnosis is made by histologic evaluation. Lipoblastoma appears as immature fat cells in varying stages of maturity with septa separating them into lobules. There should not be nuclear atypia.⁵ The histologic differential diagnosis includes other adipose tumors, most chiefly myxoid liposarcoma. These tumors have a lobular pattern without prominent septae and contain nuclear atypia with atypical mitotic figures. Myxoid liposarcoma has an infiltrating pattern similar to lipoblastomatosis and has a metastatic rate up to 60%.⁶ Imaging studies such as MRI are helpful in diagnosis, particularly in head and neck or visceral cases.³ The treatment of choice of lipoblastoma is wide excision. With complete removal, tumors rarely recur. Recurrences are more common in lipoblastomatosis or with incompletely excised primary lesions.³ A 14% to 24% recurrence rate has been recognized. Cytogenic analysis of lipomatous tumors has begun to reveal translocations in chromosome 8q11-13 region breakpoint abnormalities and translocations, specifically involving the pleomorphic adenoma gene 1, PLAG1, as the oncogenic target in lipoblastoma.⁶ Identification of these molecular mutations may provide aid in differentiating histologically similar-appearing tumors in the future.

This case illustrates a rare benign childhood tumor that can be difficult to diagnose prior to histologic examination. Our patient did not fit the typical description of a lipoblastoma, as his tumor was axially located as opposed to the more common peripheral presentation. We aim to raise awareness of this diagnosis as more cases are being recognized.

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To improve patient care and outcomes, leading dermatologists from the Cutis Editorial Board answered 5 questions on nail care. Here’s what we found.

Do you routinely perform diagnostic testing before treating for onychomycosis?

Ninety-five percent of dermatologists perform diagnostic testing before treating onychomycosis. Of them, nearly two-thirds only test before treating with systemic antifungals, while one-third test before starting systemic or topical antifungals.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
A laboratory diagnosis of onychomycosis is an absolute necessity before treating for onychomycosis, and the vast majority of our board members are testing routinely. Diagnosis should ideally be performed before initiating both oral and topical therapy. Failure to do so may lead to incorrect treatment with progression of disease and missed diagnoses of malignancy (Lipner and Scher, 2016; Lipner and Scher, 2016).

Next page: Nail fungus

What diagnostic tests do you use to confirm the presence of a nail fungus?

More than 70% of respondents use histopathology or fungal culture to confirm the presence of a nail fungus. Direct microscopy is used by 38% and only 5% use polymerase chain reaction.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Options for diagnosis are potassium hydroxide preparation with microscopy, fungal culture, or nail plate clipping with histopathology. Polymerase chain reaction is another option that is available and covered by many insurance plans. Many of our board members use histopathology and fungal culture more often than other methods. Histopathology is advantageous for its high sensitivity and capacity to detect other nail diseases, such as nail psoriasis. A disadvantage is that the identity and viability of the infecting organism cannot be determined. While fungal culture can detect both identity and viability, the organism may take several weeks to grow and there is a high false-negative rate (Lipner and Scher, 2018 [Part 1]).

Next page: Laboratory monitoring with terbinafine

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Almost half of board members perform laboratory monitoring for patients taking terbinafine, which was reasonable prior to any published data on blood count and liver function tests in patients taking this drug. However, a new study on this topic should make us reconsider our practices. This study analyzed the rate of laboratory test abnormalities in 4985 patients taking terbinafine or griseofulvin for dermatophyte infections. Elevated alanine aminotransferase, aspartate aminotransferase, anemia, lymphopenia, and neutropenia were uncommon and similar to the baseline rates. Therefore, routine interval laboratory monitoring may be unnecessary in healthy patients taking oral terbinafine for onychomycosis (Stolmeier et al).

Next page: Biotin recommendations

Do you routinely recommend biotin to your patients?

Approximately half (52%) of dermatologists do not recommend biotin to their patients. However, 29% do recommend it for hair and nail disorders.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Biotin is an essential cofactor for mammalian carboxylase enzymes that are involved in important metabolic pathways in humans. Biotin supplementation is likely unnecessary for most individuals, as biotin intake is likely sufficient in a Western diet. There are limited data on biotin supplementation to treat dermatologic conditions, especially in patients with normal biotin levels. In addition, a recent warning issued by the US Food and Drug Administration reported that consumption of biotin may interfere with laboratory tests. Therefore, biotin should not be routinely recommended to patients without sufficient evidence that it would benefit their condition (Lipner, 2018).

Next page: Medication for onychomycosis

Which medication(s) do you prescribe most often for onychomycosis?

The top medications prescribed by dermatologists for onychomycosis were oral terbinafine (62%) and topical ciclopirox (52%), followed by oral fluconazole (29%), topical efinaconazole (24%), oral itraconazole (14%), and topical tavaborole (5%).

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Oral terbinafine is most frequently prescribed by our board, likely because it has the best efficacy, dosing regimen, and minimal potential for systemic side effects or drug-drug interactions. Efficacy with ciclopirox lacquer is quite low and the medication is difficult to apply. For toenail onychomycosis, application is daily with weekly clipping and removal and monthly debridement. Patients who are not candidates for terbinafine would likely benefit more from oral itraconazole, oral fluconazole (off label), efinaconazole, or tavaborole (Lipner and Scher, 2018 [Part II]).

Next page: More tips from derms

More Tips From Dermatologists

The dermatologists we polled had the following advice for their peers:

As with care in general in dermatology, the care and treatment of healthy nails and especially diseased nails is multifaceted and is optimized by using combination therapy. The combination of an oral antifungal and a topical that treats the local area and also provides protection and a healthy environment for nail growth and repair is ideal.—Fran E. Cook-Bolden, MD (New York, New York)

It’s important to culture for fungus before starting treatment. It seems many cultures turn out to be nondermatophytes, and terbinafine is not the best treatment.—Lawrence J. Green, MD (Washington, DC)

Nails do care. Diagnoses should be confirmed.—Richard K. Scher, MD (New York, New York)

About This Survey
The survey was fielded electronically to Cutis Editorial Board Members within the United States from October 22, 2018, to November 14, 2018. A total of 21 usable responses were received.

To improve patient care and outcomes, leading dermatologists from the Cutis Editorial Board answered 5 questions on nail care. Here’s what we found.

Do you routinely perform diagnostic testing before treating for onychomycosis?

Ninety-five percent of dermatologists perform diagnostic testing before treating onychomycosis. Of them, nearly two-thirds only test before treating with systemic antifungals, while one-third test before starting systemic or topical antifungals.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
A laboratory diagnosis of onychomycosis is an absolute necessity before treating for onychomycosis, and the vast majority of our board members are testing routinely. Diagnosis should ideally be performed before initiating both oral and topical therapy. Failure to do so may lead to incorrect treatment with progression of disease and missed diagnoses of malignancy (Lipner and Scher, 2016; Lipner and Scher, 2016).

Next page: Nail fungus

What diagnostic tests do you use to confirm the presence of a nail fungus?

More than 70% of respondents use histopathology or fungal culture to confirm the presence of a nail fungus. Direct microscopy is used by 38% and only 5% use polymerase chain reaction.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Options for diagnosis are potassium hydroxide preparation with microscopy, fungal culture, or nail plate clipping with histopathology. Polymerase chain reaction is another option that is available and covered by many insurance plans. Many of our board members use histopathology and fungal culture more often than other methods. Histopathology is advantageous for its high sensitivity and capacity to detect other nail diseases, such as nail psoriasis. A disadvantage is that the identity and viability of the infecting organism cannot be determined. While fungal culture can detect both identity and viability, the organism may take several weeks to grow and there is a high false-negative rate (Lipner and Scher, 2018 [Part 1]).

Next page: Laboratory monitoring with terbinafine

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Almost half of board members perform laboratory monitoring for patients taking terbinafine, which was reasonable prior to any published data on blood count and liver function tests in patients taking this drug. However, a new study on this topic should make us reconsider our practices. This study analyzed the rate of laboratory test abnormalities in 4985 patients taking terbinafine or griseofulvin for dermatophyte infections. Elevated alanine aminotransferase, aspartate aminotransferase, anemia, lymphopenia, and neutropenia were uncommon and similar to the baseline rates. Therefore, routine interval laboratory monitoring may be unnecessary in healthy patients taking oral terbinafine for onychomycosis (Stolmeier et al).

Next page: Biotin recommendations

Do you routinely recommend biotin to your patients?

Approximately half (52%) of dermatologists do not recommend biotin to their patients. However, 29% do recommend it for hair and nail disorders.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Biotin is an essential cofactor for mammalian carboxylase enzymes that are involved in important metabolic pathways in humans. Biotin supplementation is likely unnecessary for most individuals, as biotin intake is likely sufficient in a Western diet. There are limited data on biotin supplementation to treat dermatologic conditions, especially in patients with normal biotin levels. In addition, a recent warning issued by the US Food and Drug Administration reported that consumption of biotin may interfere with laboratory tests. Therefore, biotin should not be routinely recommended to patients without sufficient evidence that it would benefit their condition (Lipner, 2018).

Next page: Medication for onychomycosis

Which medication(s) do you prescribe most often for onychomycosis?

The top medications prescribed by dermatologists for onychomycosis were oral terbinafine (62%) and topical ciclopirox (52%), followed by oral fluconazole (29%), topical efinaconazole (24%), oral itraconazole (14%), and topical tavaborole (5%).

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Oral terbinafine is most frequently prescribed by our board, likely because it has the best efficacy, dosing regimen, and minimal potential for systemic side effects or drug-drug interactions. Efficacy with ciclopirox lacquer is quite low and the medication is difficult to apply. For toenail onychomycosis, application is daily with weekly clipping and removal and monthly debridement. Patients who are not candidates for terbinafine would likely benefit more from oral itraconazole, oral fluconazole (off label), efinaconazole, or tavaborole (Lipner and Scher, 2018 [Part II]).

Next page: More tips from derms

More Tips From Dermatologists

The dermatologists we polled had the following advice for their peers:

As with care in general in dermatology, the care and treatment of healthy nails and especially diseased nails is multifaceted and is optimized by using combination therapy. The combination of an oral antifungal and a topical that treats the local area and also provides protection and a healthy environment for nail growth and repair is ideal.—Fran E. Cook-Bolden, MD (New York, New York)

It’s important to culture for fungus before starting treatment. It seems many cultures turn out to be nondermatophytes, and terbinafine is not the best treatment.—Lawrence J. Green, MD (Washington, DC)

Nails do care. Diagnoses should be confirmed.—Richard K. Scher, MD (New York, New York)

About This Survey
The survey was fielded electronically to Cutis Editorial Board Members within the United States from October 22, 2018, to November 14, 2018. A total of 21 usable responses were received.

To improve patient care and outcomes, leading dermatologists from the Cutis Editorial Board answered 5 questions on nail care. Here’s what we found.

Do you routinely perform diagnostic testing before treating for onychomycosis?

Ninety-five percent of dermatologists perform diagnostic testing before treating onychomycosis. Of them, nearly two-thirds only test before treating with systemic antifungals, while one-third test before starting systemic or topical antifungals.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
A laboratory diagnosis of onychomycosis is an absolute necessity before treating for onychomycosis, and the vast majority of our board members are testing routinely. Diagnosis should ideally be performed before initiating both oral and topical therapy. Failure to do so may lead to incorrect treatment with progression of disease and missed diagnoses of malignancy (Lipner and Scher, 2016; Lipner and Scher, 2016).

Next page: Nail fungus

What diagnostic tests do you use to confirm the presence of a nail fungus?

More than 70% of respondents use histopathology or fungal culture to confirm the presence of a nail fungus. Direct microscopy is used by 38% and only 5% use polymerase chain reaction.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Options for diagnosis are potassium hydroxide preparation with microscopy, fungal culture, or nail plate clipping with histopathology. Polymerase chain reaction is another option that is available and covered by many insurance plans. Many of our board members use histopathology and fungal culture more often than other methods. Histopathology is advantageous for its high sensitivity and capacity to detect other nail diseases, such as nail psoriasis. A disadvantage is that the identity and viability of the infecting organism cannot be determined. While fungal culture can detect both identity and viability, the organism may take several weeks to grow and there is a high false-negative rate (Lipner and Scher, 2018 [Part 1]).

Next page: Laboratory monitoring with terbinafine

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Almost half of board members perform laboratory monitoring for patients taking terbinafine, which was reasonable prior to any published data on blood count and liver function tests in patients taking this drug. However, a new study on this topic should make us reconsider our practices. This study analyzed the rate of laboratory test abnormalities in 4985 patients taking terbinafine or griseofulvin for dermatophyte infections. Elevated alanine aminotransferase, aspartate aminotransferase, anemia, lymphopenia, and neutropenia were uncommon and similar to the baseline rates. Therefore, routine interval laboratory monitoring may be unnecessary in healthy patients taking oral terbinafine for onychomycosis (Stolmeier et al).

Next page: Biotin recommendations

Do you routinely recommend biotin to your patients?

Approximately half (52%) of dermatologists do not recommend biotin to their patients. However, 29% do recommend it for hair and nail disorders.

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Biotin is an essential cofactor for mammalian carboxylase enzymes that are involved in important metabolic pathways in humans. Biotin supplementation is likely unnecessary for most individuals, as biotin intake is likely sufficient in a Western diet. There are limited data on biotin supplementation to treat dermatologic conditions, especially in patients with normal biotin levels. In addition, a recent warning issued by the US Food and Drug Administration reported that consumption of biotin may interfere with laboratory tests. Therefore, biotin should not be routinely recommended to patients without sufficient evidence that it would benefit their condition (Lipner, 2018).

Next page: Medication for onychomycosis

Which medication(s) do you prescribe most often for onychomycosis?

The top medications prescribed by dermatologists for onychomycosis were oral terbinafine (62%) and topical ciclopirox (52%), followed by oral fluconazole (29%), topical efinaconazole (24%), oral itraconazole (14%), and topical tavaborole (5%).

Expert Commentary
Provided by Shari R. Lipner, MD, PhD (New York, New York)
Oral terbinafine is most frequently prescribed by our board, likely because it has the best efficacy, dosing regimen, and minimal potential for systemic side effects or drug-drug interactions. Efficacy with ciclopirox lacquer is quite low and the medication is difficult to apply. For toenail onychomycosis, application is daily with weekly clipping and removal and monthly debridement. Patients who are not candidates for terbinafine would likely benefit more from oral itraconazole, oral fluconazole (off label), efinaconazole, or tavaborole (Lipner and Scher, 2018 [Part II]).

Next page: More tips from derms

More Tips From Dermatologists

The dermatologists we polled had the following advice for their peers:

As with care in general in dermatology, the care and treatment of healthy nails and especially diseased nails is multifaceted and is optimized by using combination therapy. The combination of an oral antifungal and a topical that treats the local area and also provides protection and a healthy environment for nail growth and repair is ideal.—Fran E. Cook-Bolden, MD (New York, New York)

It’s important to culture for fungus before starting treatment. It seems many cultures turn out to be nondermatophytes, and terbinafine is not the best treatment.—Lawrence J. Green, MD (Washington, DC)

Nails do care. Diagnoses should be confirmed.—Richard K. Scher, MD (New York, New York)

About This Survey
The survey was fielded electronically to Cutis Editorial Board Members within the United States from October 22, 2018, to November 14, 2018. A total of 21 usable responses were received.

Given its limited systemic toxicity, topical anthralin is an acceptable second-line treatment option for pediatric alopecia areata (AA), according to Sean Z. Wu, MD, of the department of dermatology, University of Cincinnati, and his associates.

In a retrospective study of 37 pediatric patients with AA, published in Pediatric Dermatology, Dr. Wu and his colleagues found that almost two-thirds experienced at least 50% regrowth of hair with topical anthralin treatment, but they described severe dermatitis and relapses as “potential drawbacks” of treatment.

The 37 patients were in the Cleveland Clinic AA areata database and began treatment with anthralin between 2004 and 2015, at aged 2-17 years (mean age 9). Over half (22) were females and most (31) were white. About 65% had patchy AA; the remainder had either alopecia totalis or alopecia universalis. Prior treatments included topical corticosteroids, minoxidil, and intralesional corticosteroids; four patients had not been treated previously. Patients were followed up from 51 days to more than 10 years, with a mean duration of 2.5 years. Treatment regimens, titrated up to achieve a mild to moderate dermatitis, included application of 0.5% cream for 5 minutes twice a week up to 1.0% cream for 30 minutes a day.

With topical anthralin, 12 (32%) of patients had complete scalp regrowth, 25 (68%) experienced at least 50% regrowth, and 5 (14%) had no response; in five patients, no follow-up information was available. Among those with at least 50% regrowth, the initial response was first noted at a mean of 3.4 months, and the mean time to maximal response was 15 months. This timeline suggests that treatment with anthralin should be continued beyond 1 year to ensure maximum beneficial results with hair regrowth, the authors wrote.

Factors associated with a positive response to treatment included less than 50% of scalp involvement. The two patients who used anthralin as monotherapy did not achieve a 50% scalp response, but the four treatment-naive patients were among those with at least 50% scalp regrowth, versus three of the five patients (60%) who had been treated previously with systemic steroids.

Two potential clinical limitations were noted during the study. Four patients had to stop treatment because of dermatitis, which suggests that patients and parents should be counseled about the potential for severe skin irritation with this treatment, the authors said. And among those who achieved at least 50% scalp regrowth, 16 of the 25 (64%) relapsed. The authors speculated that the effects of the drug could be temporary “or that the disease process may be able to overcome the anthralin effect over time.”

Dr. Wu and his coauthors cited the retrospective design and the small population size as major limitations of the study. Because some patients continued other treatments, it is “difficult to attribute scalp regrowth entirely to anthralin,” and variations in formulation and in treatment regimens are also factors to be considered, they cautioned. That AA has been found to spontaneously resolve, depending upon the severity of the disease, presents additional challenges for clinicians attempting to determine the extent to which anthralin offers therapeutic benefit, they pointed out.

In spite of the drug’s limitations, the authors concluded that the treatment was a safe and useful option as an “adjunct for those who fail first-line therapy with topical or intralesional corticosteroids.” They added that more work is needed to tailor treatment formulation, frequency, and duration to the specific needs of pediatric patients.

The authors had no relevant financial disclosures to report.

SOURCE: Wu SZ et al. Pediatr Dermatol. 2018;35:817-20.

Given its limited systemic toxicity, topical anthralin is an acceptable second-line treatment option for pediatric alopecia areata (AA), according to Sean Z. Wu, MD, of the department of dermatology, University of Cincinnati, and his associates.

In a retrospective study of 37 pediatric patients with AA, published in Pediatric Dermatology, Dr. Wu and his colleagues found that almost two-thirds experienced at least 50% regrowth of hair with topical anthralin treatment, but they described severe dermatitis and relapses as “potential drawbacks” of treatment.

The 37 patients were in the Cleveland Clinic AA areata database and began treatment with anthralin between 2004 and 2015, at aged 2-17 years (mean age 9). Over half (22) were females and most (31) were white. About 65% had patchy AA; the remainder had either alopecia totalis or alopecia universalis. Prior treatments included topical corticosteroids, minoxidil, and intralesional corticosteroids; four patients had not been treated previously. Patients were followed up from 51 days to more than 10 years, with a mean duration of 2.5 years. Treatment regimens, titrated up to achieve a mild to moderate dermatitis, included application of 0.5% cream for 5 minutes twice a week up to 1.0% cream for 30 minutes a day.

With topical anthralin, 12 (32%) of patients had complete scalp regrowth, 25 (68%) experienced at least 50% regrowth, and 5 (14%) had no response; in five patients, no follow-up information was available. Among those with at least 50% regrowth, the initial response was first noted at a mean of 3.4 months, and the mean time to maximal response was 15 months. This timeline suggests that treatment with anthralin should be continued beyond 1 year to ensure maximum beneficial results with hair regrowth, the authors wrote.

Factors associated with a positive response to treatment included less than 50% of scalp involvement. The two patients who used anthralin as monotherapy did not achieve a 50% scalp response, but the four treatment-naive patients were among those with at least 50% scalp regrowth, versus three of the five patients (60%) who had been treated previously with systemic steroids.

Two potential clinical limitations were noted during the study. Four patients had to stop treatment because of dermatitis, which suggests that patients and parents should be counseled about the potential for severe skin irritation with this treatment, the authors said. And among those who achieved at least 50% scalp regrowth, 16 of the 25 (64%) relapsed. The authors speculated that the effects of the drug could be temporary “or that the disease process may be able to overcome the anthralin effect over time.”

Dr. Wu and his coauthors cited the retrospective design and the small population size as major limitations of the study. Because some patients continued other treatments, it is “difficult to attribute scalp regrowth entirely to anthralin,” and variations in formulation and in treatment regimens are also factors to be considered, they cautioned. That AA has been found to spontaneously resolve, depending upon the severity of the disease, presents additional challenges for clinicians attempting to determine the extent to which anthralin offers therapeutic benefit, they pointed out.

In spite of the drug’s limitations, the authors concluded that the treatment was a safe and useful option as an “adjunct for those who fail first-line therapy with topical or intralesional corticosteroids.” They added that more work is needed to tailor treatment formulation, frequency, and duration to the specific needs of pediatric patients.

The authors had no relevant financial disclosures to report.

SOURCE: Wu SZ et al. Pediatr Dermatol. 2018;35:817-20.

Given its limited systemic toxicity, topical anthralin is an acceptable second-line treatment option for pediatric alopecia areata (AA), according to Sean Z. Wu, MD, of the department of dermatology, University of Cincinnati, and his associates.

In a retrospective study of 37 pediatric patients with AA, published in Pediatric Dermatology, Dr. Wu and his colleagues found that almost two-thirds experienced at least 50% regrowth of hair with topical anthralin treatment, but they described severe dermatitis and relapses as “potential drawbacks” of treatment.

The 37 patients were in the Cleveland Clinic AA areata database and began treatment with anthralin between 2004 and 2015, at aged 2-17 years (mean age 9). Over half (22) were females and most (31) were white. About 65% had patchy AA; the remainder had either alopecia totalis or alopecia universalis. Prior treatments included topical corticosteroids, minoxidil, and intralesional corticosteroids; four patients had not been treated previously. Patients were followed up from 51 days to more than 10 years, with a mean duration of 2.5 years. Treatment regimens, titrated up to achieve a mild to moderate dermatitis, included application of 0.5% cream for 5 minutes twice a week up to 1.0% cream for 30 minutes a day.

With topical anthralin, 12 (32%) of patients had complete scalp regrowth, 25 (68%) experienced at least 50% regrowth, and 5 (14%) had no response; in five patients, no follow-up information was available. Among those with at least 50% regrowth, the initial response was first noted at a mean of 3.4 months, and the mean time to maximal response was 15 months. This timeline suggests that treatment with anthralin should be continued beyond 1 year to ensure maximum beneficial results with hair regrowth, the authors wrote.

Factors associated with a positive response to treatment included less than 50% of scalp involvement. The two patients who used anthralin as monotherapy did not achieve a 50% scalp response, but the four treatment-naive patients were among those with at least 50% scalp regrowth, versus three of the five patients (60%) who had been treated previously with systemic steroids.

Two potential clinical limitations were noted during the study. Four patients had to stop treatment because of dermatitis, which suggests that patients and parents should be counseled about the potential for severe skin irritation with this treatment, the authors said. And among those who achieved at least 50% scalp regrowth, 16 of the 25 (64%) relapsed. The authors speculated that the effects of the drug could be temporary “or that the disease process may be able to overcome the anthralin effect over time.”

Dr. Wu and his coauthors cited the retrospective design and the small population size as major limitations of the study. Because some patients continued other treatments, it is “difficult to attribute scalp regrowth entirely to anthralin,” and variations in formulation and in treatment regimens are also factors to be considered, they cautioned. That AA has been found to spontaneously resolve, depending upon the severity of the disease, presents additional challenges for clinicians attempting to determine the extent to which anthralin offers therapeutic benefit, they pointed out.

In spite of the drug’s limitations, the authors concluded that the treatment was a safe and useful option as an “adjunct for those who fail first-line therapy with topical or intralesional corticosteroids.” They added that more work is needed to tailor treatment formulation, frequency, and duration to the specific needs of pediatric patients.

The authors had no relevant financial disclosures to report.

SOURCE: Wu SZ et al. Pediatr Dermatol. 2018;35:817-20.

A KOH (potassium hydroxide) test done at the visit was negative as well as a fungal culture of each toenail.

The patient was diagnosed with congenital malalignment of the great toenails (CMGTN) based on history and morphologic appearance.

Congenital malalignment of the great toenails is an underrecognized and underreported nail disorder characterized by lateral deviation of the nail plate, which is not parallel to the longitudinal axis of the distal phalanx.¹ The cause is unknown. Some reports suggest a genetic cause being transmitted in an autosomal dominant fashion with variable expression.² There have been reports of CMGTN in monozygotic and dizygotic twins making this theory likely.³ Other authors consider an external cause such as amniotic bands, neonatal asphyxia, vascular malformations, and uterine pressure. This condition also has been reported in patients with Rubinstein-Taybi syndrome.⁴

The nail changes can occur at birth but in some cases, such as our patient, the nails become dystrophic months to years after birth. Characteristic nail changes include shorter, discolored, hyperkeratotic nails with transverse groove or ridges. In some cases, the dystrophic nails may cause inflammation and tenderness and is the most common cause of ingrown toenails in children.

The differential diagnosis includes onychomycosis, traumatic nails, nail psoriasis, pachyonychia congenital (PC), and onychomadesis. Onychomycosis can present with white or yellow discoloration of the nail that in some cases can be associated with nail breakage, hyperkeratosis, onycholysis, and subungual debris. Either fungal culture or periodic acid shift stain of nail clippings can help confirm or exclude this diagnosis. Psoriatic nails present with nail pits, oils spots, and onycholysis. Traumatic nail changes may occur from using small shoes and trauma from running or playing soccer, and presents with subungual hemorrhage and nail dystrophy of the first or second toenail. PC is a genetic disorder caused by a mutation in certain keratin proteins of the skin (k6a, k6b, K16 and K17). These patients usually have other skin findings including palmoplantar keratoderma, white plaques on the mouth, and skin cysts (steatocystoma multiplex and vellus hair cysts). Nail changes characteristic of PC includes subungual hyperkeratosis that causes a wedge shape thickening of the nail bed (pincer nails).⁵ Onychomadesis can be seen after viral infections such as hand-foot-mouth disease or in patients taking chemotherapy drugs that affect nail growth.

CMGTN usually resolves with time, but some patients with severe deviation and paronychia may need surgical correction.⁶
 

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. Email her at [email protected].

References

1. Dermatol Online J. 2014 Jan 15;20(1):21251.

2. J Dtsch Dermatol Ges. 2012 May;10(5):326-30.

3. J Am Acad Dermatol. 2007 Oct;57(4):711-5.

4. Pediatr Dermatol. 2004 Jan-Feb;21(1):44-7.

5. Curr Opin Pediatr. 2014 Aug;26(4):440-5.

6. Skin Appendage Disord. 2018 Oct;4(4):230-5.

A KOH (potassium hydroxide) test done at the visit was negative as well as a fungal culture of each toenail.

The patient was diagnosed with congenital malalignment of the great toenails (CMGTN) based on history and morphologic appearance.

Congenital malalignment of the great toenails is an underrecognized and underreported nail disorder characterized by lateral deviation of the nail plate, which is not parallel to the longitudinal axis of the distal phalanx.¹ The cause is unknown. Some reports suggest a genetic cause being transmitted in an autosomal dominant fashion with variable expression.² There have been reports of CMGTN in monozygotic and dizygotic twins making this theory likely.³ Other authors consider an external cause such as amniotic bands, neonatal asphyxia, vascular malformations, and uterine pressure. This condition also has been reported in patients with Rubinstein-Taybi syndrome.⁴

The nail changes can occur at birth but in some cases, such as our patient, the nails become dystrophic months to years after birth. Characteristic nail changes include shorter, discolored, hyperkeratotic nails with transverse groove or ridges. In some cases, the dystrophic nails may cause inflammation and tenderness and is the most common cause of ingrown toenails in children.

The differential diagnosis includes onychomycosis, traumatic nails, nail psoriasis, pachyonychia congenital (PC), and onychomadesis. Onychomycosis can present with white or yellow discoloration of the nail that in some cases can be associated with nail breakage, hyperkeratosis, onycholysis, and subungual debris. Either fungal culture or periodic acid shift stain of nail clippings can help confirm or exclude this diagnosis. Psoriatic nails present with nail pits, oils spots, and onycholysis. Traumatic nail changes may occur from using small shoes and trauma from running or playing soccer, and presents with subungual hemorrhage and nail dystrophy of the first or second toenail. PC is a genetic disorder caused by a mutation in certain keratin proteins of the skin (k6a, k6b, K16 and K17). These patients usually have other skin findings including palmoplantar keratoderma, white plaques on the mouth, and skin cysts (steatocystoma multiplex and vellus hair cysts). Nail changes characteristic of PC includes subungual hyperkeratosis that causes a wedge shape thickening of the nail bed (pincer nails).⁵ Onychomadesis can be seen after viral infections such as hand-foot-mouth disease or in patients taking chemotherapy drugs that affect nail growth.

CMGTN usually resolves with time, but some patients with severe deviation and paronychia may need surgical correction.⁶
 

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. Email her at [email protected].

References

1. Dermatol Online J. 2014 Jan 15;20(1):21251.

2. J Dtsch Dermatol Ges. 2012 May;10(5):326-30.

3. J Am Acad Dermatol. 2007 Oct;57(4):711-5.

4. Pediatr Dermatol. 2004 Jan-Feb;21(1):44-7.

5. Curr Opin Pediatr. 2014 Aug;26(4):440-5.

6. Skin Appendage Disord. 2018 Oct;4(4):230-5.

A KOH (potassium hydroxide) test done at the visit was negative as well as a fungal culture of each toenail.

The patient was diagnosed with congenital malalignment of the great toenails (CMGTN) based on history and morphologic appearance.

Congenital malalignment of the great toenails is an underrecognized and underreported nail disorder characterized by lateral deviation of the nail plate, which is not parallel to the longitudinal axis of the distal phalanx.¹ The cause is unknown. Some reports suggest a genetic cause being transmitted in an autosomal dominant fashion with variable expression.² There have been reports of CMGTN in monozygotic and dizygotic twins making this theory likely.³ Other authors consider an external cause such as amniotic bands, neonatal asphyxia, vascular malformations, and uterine pressure. This condition also has been reported in patients with Rubinstein-Taybi syndrome.⁴

The nail changes can occur at birth but in some cases, such as our patient, the nails become dystrophic months to years after birth. Characteristic nail changes include shorter, discolored, hyperkeratotic nails with transverse groove or ridges. In some cases, the dystrophic nails may cause inflammation and tenderness and is the most common cause of ingrown toenails in children.

The differential diagnosis includes onychomycosis, traumatic nails, nail psoriasis, pachyonychia congenital (PC), and onychomadesis. Onychomycosis can present with white or yellow discoloration of the nail that in some cases can be associated with nail breakage, hyperkeratosis, onycholysis, and subungual debris. Either fungal culture or periodic acid shift stain of nail clippings can help confirm or exclude this diagnosis. Psoriatic nails present with nail pits, oils spots, and onycholysis. Traumatic nail changes may occur from using small shoes and trauma from running or playing soccer, and presents with subungual hemorrhage and nail dystrophy of the first or second toenail. PC is a genetic disorder caused by a mutation in certain keratin proteins of the skin (k6a, k6b, K16 and K17). These patients usually have other skin findings including palmoplantar keratoderma, white plaques on the mouth, and skin cysts (steatocystoma multiplex and vellus hair cysts). Nail changes characteristic of PC includes subungual hyperkeratosis that causes a wedge shape thickening of the nail bed (pincer nails).⁵ Onychomadesis can be seen after viral infections such as hand-foot-mouth disease or in patients taking chemotherapy drugs that affect nail growth.

CMGTN usually resolves with time, but some patients with severe deviation and paronychia may need surgical correction.⁶
 

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. Email her at [email protected].

References

1. Dermatol Online J. 2014 Jan 15;20(1):21251.

2. J Dtsch Dermatol Ges. 2012 May;10(5):326-30.

3. J Am Acad Dermatol. 2007 Oct;57(4):711-5.

4. Pediatr Dermatol. 2004 Jan-Feb;21(1):44-7.

5. Curr Opin Pediatr. 2014 Aug;26(4):440-5.

6. Skin Appendage Disord. 2018 Oct;4(4):230-5.

The etiology of central centrifugal cicatricial alopecia (CCCA), a clinical and histological pattern of hair loss on the central scalp, has been well studied. This disease is chronic and progressive, with extensive follicular destruction and eventual burnout.^1,2 Central centrifugal cicatricial alopecia is most commonly seen in patients of African descent and has been shown to be 1 of the 5 most common dermatologic diagnoses in black patients.^3,4 The top 5 dermatologic diagnoses within this population include acne vulgaris (28.4%), dyschromia (19.9%), eczema (9.1%), alopecia (8.3%), and seborrheic dermatitis (6.7%).⁴ The incidence rate of CCCA is estimated to be 5.6%.^3,5 Most patients are women, with onset between the second and fourth decades of life.⁶

Central centrifugal cicatricial alopecia treatment efficacy is inversely correlated with disease duration. The primary goal of treatment is to prevent progression. Efforts are made to stimulate regrowth in areas that are not permanently scarred. When patients present with a substantial amount of scarring hair loss, dermatologists often are limited in their ability to achieve a cosmetically acceptable pattern of growth. Generally, hair is connected to a sense of self-worth in black women, and any type of hair loss has been shown to lead to frustration and decreased self-esteem.⁷ A 1994 study showed that 75% (44/58) of women with androgenetic alopecia had decreased self-esteem and 50% (29/58) had social challenges.⁸

The purpose of this pilot study was to determine the personal, historical, logistical, or environmental factors that preclude women from obtaining medical care for CCCA and to investigate how CCCA affects quality of life (QOL) and psychological well-being.

Methods

The investigators designed a survey study of adult, English-speaking, black women diagnosed with CCCA at the Northwestern University Department of Dermatology (Chicago, Illinois) between 2011 and 2017. Patients were selected from the electronic data warehouse compiled by the Department of Dermatology and were included if they fulfilled the following criteria: evaluated in the dermatology department between September 1, 2011, and September 30, 2017, by any faculty physician; diagnosed with CCCA; and aged 18 years or older. Patients were excluded if they did not speak English, as interpreters were not available. All patients who fulfilled the inclusion criteria provided signed informed consent prior to participation. All surveys were disseminated in the office or via telephone from fall 2016 to spring 2017 and took 10 to 15 minutes to complete. The research was approved by the authors’ institutional review board (IRB ID STU00203449).

Survey Instrument
The CCCA Barriers to Care and Quality of Life Survey is a 53-item survey instrument created by the study’s authors to measure 2 aspects of CCCA hair loss: barriers to medical care and QOL. The initial set of questions pertained to initial hair loss discovery and the number of physicians, both dermatologist and otherwise, seen for hair evaluation. Patients then rated their physician interactions on an ordinal Likert scale (1=poor, 2=fair, 3=good, 4=very good, 5=excellent). If patients saw more than 1 dermatologist or nondermatologist physician, ratings for each provider were included in our analysis. Patients listed the top 3 factors they considered when seeking medical care for their hair loss in order of importance. They also indicated how much they agreed with QOL statements on a Likert scale (1=strongly disagree, 2=disagree, 3=neutral, 4=agree, 5=strongly agree).

Data Analysis
Analyses were completed using data analysis software JMP Pro 13 from SAS and a Microsoft Excel spreadsheet. Continuous data were presented as mean, SD, median, minimum, and maximum. Categorical data were presented as counts and percentages. Nine QOL items were aggregated into a self-esteem category (questions 30–38).

Cronbach α, a statistical measure of internal consistency and how closely related items are in a group, was used to evaluate internal consistency reliability; values of 0.70 or greater indicate acceptable reliability.

Results

Of 501 individuals contacted, 34 completed the survey (7% completion rate). Nonrespondents included 7 who refused to participate and 460 who could not be contacted. All respondents self-identified as black women. Median age at time of survey administration was 46 years (range, 28–79 years); median age at CCCA diagnosis was 42 years (range, 15–73 years). Respondents did not significantly differ in age from nonrespondents (P=.46). The majority of respondents had an associate’s degree, bachelor’s degree, or advanced degree of education (master of arts, doctor of medicine, doctor of jurisprudence, doctor of philosophy); however, 8 women reported completing some college, 1 reported completing high school, and 1 reported no schooling. Three respondents had no health insurance.

Initial Hair Loss Discovery
The majority of respondents (22/34 [65%]) were first to notice their hair loss, while 5 (15%) reported hairstylists as the initial observers. Twelve respondents (35%) initially went to a physician to learn why they were losing hair; 6 (18%) instead utilized hairstylists or the Internet. Fifteen women (44%) waited more than 1 month up to 6 months after noticing hair loss before seeing a physician instead of going immediately within a 4-week period, and 16 (47%) waited 1 year or more.

Nondermatologist Consultation
Almost half (16/34 [47%]) of the women went to a nondermatologist physician regarding their hair loss; of them, half (8/16 [50%]) reported their physician did not examine the scalp, 3 (19%) reported their physician offered a biopsy, and none of them reported that their physician diagnosed them with CCCA. The median patient rating of their nondermatologist physician interactions was good (3 on a 5-point scale). Table 1 and Figure 1 show responses to individual items.

Dermatologist Consultation
All 34 respondents presented to a dermatologist. The majority of respondents (22/34 [65%]) saw either 1 or 2 dermatologists for their hair loss. Three (9%) reported their dermatologist did not examine their scalp. Twelve respondents (35%) reported their dermatologist did not offer a biopsy. Twenty-one respondents (62%) reported a CCCA diagnosis from the first dermatologist they saw. Twenty-three respondents (68%) were diagnosed by dermatologists with expertise in hair disorders. Sixteen (47%) were diagnosed by dermatologists within a skin-of-color center. Fourteen (41%) initial dermatology consultations were race concordant.

The median patient rating of their dermatologist interactions was excellent (5 on a 5-point scale). Table 2 and Figure 2 show responses to individual items. Respondents saw an average of 3 different providers, both dermatologists and otherwise.

Waiting to See a Dermatologist
Nearly all respondents (31/34 [91%]) recommended that other women with hair loss immediately go see a dermatologist.

Barriers to Care
The top 5 factors reported as most important when initially seeking care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Table 3 shows frequency counts for these freely reported factors.

Quality of Life
The median score on 9 aggregated self-esteem items was 4 on a 5-point scale, representing an agree response to statements such as “I feel embarrassed, self-conscious, or frustrated about my hair loss” (28/34 [82%]) and “My hair loss bothers me” (28/34 [82%])(Table 4). Cronbach α for self-esteem survey items was 0.7826.

For the nonaggregated items, many respondents strongly disagreed with statements pertaining to activities of daily living, including “I take care of where I sit or stand at social gatherings due to my hair loss” (18/34 [53%]), “My hair loss makes it difficult for me to go to the grocery store” (29/34 [85%]), “My hair loss makes it difficult for me to attend faith-based activities” (30/34 [88%]), “My hair loss makes it difficult for me to exercise” (23/34 [68%]), “My hair loss makes it difficult for me to go to work and/or school” (24/34 [71%]), “My hair loss makes it difficult for me to go out with a significant other” (24/34 [71%]), “My hair loss makes it difficult for me to spend time with family” (27/34 [79%]), and “My hair loss makes it difficult for me to go to a hairstylist” (16/34 [47%]).

Comment

The majority of respondents were first to discover their hair loss. Harbingers of CCCA hair loss include paresthesia, tenderness, and itch,⁶ symptoms that are hard to ignore. Unfortunately, many patients notice hair thinning years after the scarring process has begun and a notable amount of hair has already been lost.^6,9

Fifteen percent of respondents learned about their hair loss from their hairstylist. Women of African descent often maintain hairstyles that require frequent interactions with a hair care professional.^7,10 As a result, hairstylists are at the forefront of early alopecia detection and are a valued resource in the black community. Open dialogue between dermatologists and hair care professionals could funnel women with hair loss into treatment before extensive damage.

Fifteen women (44%) recalled a waiting period of several months before seeking medical assistance, and 16 (47%) reported waiting 1 year or more. However, 91% of respondents indicated that women with hair loss should immediately see a physician for evaluation, thus patient experiences underscore the importance of early treatment. In our experience, many patients wait years before presenting to a physician. Some work with their hairstylists first to address the issue, while others do not realize how notable the loss has become. Some have a negative experience with one provider or are told there is nothing that can be done and then wait many years to see a second provider. Proper education of patients, physicians, and hairstylists is important in the identification and prompt treatment of this condition.

It is perhaps to be expected that patients rated interactions with dermatologists as excellent and very good more frequently than interactions with nondermatologists, which may be due to an absence of thorough hair evaluation with nondermatologists. Respondents reported that only half of nondermatologist providers actually examined their scalp during an initial encounter. However, both physician groups had the lowest frequencies of excellent and very good ratings on “understanding of your hair” (Tables 1 and 2). Patients with hair loss seek immediate answers, and often it is the specialist that can give them a firm diagnosis as opposed to a primary care provider. The fact that dermatologists and nondermatologists alike scored poorly on patient-perceived understanding of CCCA indicates an area for improvement within patient-physician interactions and physician knowledge.

The top 5 factors important to respondents when obtaining medical care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Patients with CCCA seeing dermatologists may discern a lack of experience with ethnic hair that leads patients to doubt their physicians’ ability to provide adequate care and decreased shared decision-making.^11,12 These patient perceptions are not unfounded; a 2008 study showed that dermatology residents are not uniformly trained in diseases pertaining to patients with skin of color.¹³ Thus, incorporation of education on skin of color in dermatology training programs is critical.

Finally, hair loss patients often have concerns regarding how medical therapeutics could adversely affect personal hair care regimens, including washing and hairstyling practices. Current research demonstrates that patients consider treatment effectiveness and ability to be integrated into daily routines after establishing medical care.¹⁴ The present study shows that some CCCA patients contemplate how well a therapy will work before seeking medical care, demonstrating that patients continue to have these concerns after establishing medical care. Consideration of treatment effectiveness is important for both patients and providers, as there is minimal evidence behind current CCCA management practices. The ability for treatments to be easily integrated into daily hair care habits is important to maintain patient compliance.

Participants’ median self-esteem scores indicate the effect of CCCA on morale and self-perception. Items scrutinizing this construct had acceptable internal consistency reliability. It is interesting to note that activities of daily living were not impacted by hair loss. Examination of self-esteem is important in the alopecia population because the effect of hair loss on mental status is well documented.^15-17 Low self-esteem has been reported as a prospective risk factor for clinical depression.^18-20 In black patients, clinical depression rates surpass those of Hispanics and non-Hispanic white individuals.²¹ Dermatologists must consider the psychological status of all patients, particularly populations at risk for severe disease.

Limitations of this study include the small (34 participants) and mostly highly educated sample size, limited survey validity, and potential patient bias. Because many patients changed their address and/or telephone number in the time between CCCA diagnosis and the present study, we were left with a small pilot study, which minimizes the impact of our findings. Furthermore, our survey was created by a single expert’s opinion and modeling from preexisting alopecia questionnaires¹⁶; full validity procedures analyzing face, content, and criterion validity were not undertaken. Finally, the majority of respondents were patients of one of the study’s authors (S.S.L.P.), which could influence survey responses. The fact that some providers were hair experts and some were race concordant with their patients also could potentially affect the responses received, which was not analyzed in the present study. Future studies with more respondents from multiple providers would help clarify our preliminary findings.

Conclusion

Analysis of barriers to care and QOL in patients with skin of color is an essential addition to dermatologic discourse. Alopecia is particularly important to investigate, as prior research has found it to be one of the top 5 diagnoses made in patients with skin of color.^3,4 Alopecia has been shown to negatively affect QOL.^15,22,23 This study, although limited by small sample size, suggests CCCA also is a contributor to self-esteem challenges, similar to other forms of hair loss. Patient-physician interactions and personal hairstyling practices are prominent barriers to care for CCCA patients, demonstrating the need for quality education on skin of color and cultural competency in dermatology residencies across the country.

The etiology of central centrifugal cicatricial alopecia (CCCA), a clinical and histological pattern of hair loss on the central scalp, has been well studied. This disease is chronic and progressive, with extensive follicular destruction and eventual burnout.^1,2 Central centrifugal cicatricial alopecia is most commonly seen in patients of African descent and has been shown to be 1 of the 5 most common dermatologic diagnoses in black patients.^3,4 The top 5 dermatologic diagnoses within this population include acne vulgaris (28.4%), dyschromia (19.9%), eczema (9.1%), alopecia (8.3%), and seborrheic dermatitis (6.7%).⁴ The incidence rate of CCCA is estimated to be 5.6%.^3,5 Most patients are women, with onset between the second and fourth decades of life.⁶

Central centrifugal cicatricial alopecia treatment efficacy is inversely correlated with disease duration. The primary goal of treatment is to prevent progression. Efforts are made to stimulate regrowth in areas that are not permanently scarred. When patients present with a substantial amount of scarring hair loss, dermatologists often are limited in their ability to achieve a cosmetically acceptable pattern of growth. Generally, hair is connected to a sense of self-worth in black women, and any type of hair loss has been shown to lead to frustration and decreased self-esteem.⁷ A 1994 study showed that 75% (44/58) of women with androgenetic alopecia had decreased self-esteem and 50% (29/58) had social challenges.⁸

The purpose of this pilot study was to determine the personal, historical, logistical, or environmental factors that preclude women from obtaining medical care for CCCA and to investigate how CCCA affects quality of life (QOL) and psychological well-being.

Methods

The investigators designed a survey study of adult, English-speaking, black women diagnosed with CCCA at the Northwestern University Department of Dermatology (Chicago, Illinois) between 2011 and 2017. Patients were selected from the electronic data warehouse compiled by the Department of Dermatology and were included if they fulfilled the following criteria: evaluated in the dermatology department between September 1, 2011, and September 30, 2017, by any faculty physician; diagnosed with CCCA; and aged 18 years or older. Patients were excluded if they did not speak English, as interpreters were not available. All patients who fulfilled the inclusion criteria provided signed informed consent prior to participation. All surveys were disseminated in the office or via telephone from fall 2016 to spring 2017 and took 10 to 15 minutes to complete. The research was approved by the authors’ institutional review board (IRB ID STU00203449).

Survey Instrument
The CCCA Barriers to Care and Quality of Life Survey is a 53-item survey instrument created by the study’s authors to measure 2 aspects of CCCA hair loss: barriers to medical care and QOL. The initial set of questions pertained to initial hair loss discovery and the number of physicians, both dermatologist and otherwise, seen for hair evaluation. Patients then rated their physician interactions on an ordinal Likert scale (1=poor, 2=fair, 3=good, 4=very good, 5=excellent). If patients saw more than 1 dermatologist or nondermatologist physician, ratings for each provider were included in our analysis. Patients listed the top 3 factors they considered when seeking medical care for their hair loss in order of importance. They also indicated how much they agreed with QOL statements on a Likert scale (1=strongly disagree, 2=disagree, 3=neutral, 4=agree, 5=strongly agree).

Data Analysis
Analyses were completed using data analysis software JMP Pro 13 from SAS and a Microsoft Excel spreadsheet. Continuous data were presented as mean, SD, median, minimum, and maximum. Categorical data were presented as counts and percentages. Nine QOL items were aggregated into a self-esteem category (questions 30–38).

Cronbach α, a statistical measure of internal consistency and how closely related items are in a group, was used to evaluate internal consistency reliability; values of 0.70 or greater indicate acceptable reliability.

Results

Of 501 individuals contacted, 34 completed the survey (7% completion rate). Nonrespondents included 7 who refused to participate and 460 who could not be contacted. All respondents self-identified as black women. Median age at time of survey administration was 46 years (range, 28–79 years); median age at CCCA diagnosis was 42 years (range, 15–73 years). Respondents did not significantly differ in age from nonrespondents (P=.46). The majority of respondents had an associate’s degree, bachelor’s degree, or advanced degree of education (master of arts, doctor of medicine, doctor of jurisprudence, doctor of philosophy); however, 8 women reported completing some college, 1 reported completing high school, and 1 reported no schooling. Three respondents had no health insurance.

Initial Hair Loss Discovery
The majority of respondents (22/34 [65%]) were first to notice their hair loss, while 5 (15%) reported hairstylists as the initial observers. Twelve respondents (35%) initially went to a physician to learn why they were losing hair; 6 (18%) instead utilized hairstylists or the Internet. Fifteen women (44%) waited more than 1 month up to 6 months after noticing hair loss before seeing a physician instead of going immediately within a 4-week period, and 16 (47%) waited 1 year or more.

Nondermatologist Consultation
Almost half (16/34 [47%]) of the women went to a nondermatologist physician regarding their hair loss; of them, half (8/16 [50%]) reported their physician did not examine the scalp, 3 (19%) reported their physician offered a biopsy, and none of them reported that their physician diagnosed them with CCCA. The median patient rating of their nondermatologist physician interactions was good (3 on a 5-point scale). Table 1 and Figure 1 show responses to individual items.

Dermatologist Consultation
All 34 respondents presented to a dermatologist. The majority of respondents (22/34 [65%]) saw either 1 or 2 dermatologists for their hair loss. Three (9%) reported their dermatologist did not examine their scalp. Twelve respondents (35%) reported their dermatologist did not offer a biopsy. Twenty-one respondents (62%) reported a CCCA diagnosis from the first dermatologist they saw. Twenty-three respondents (68%) were diagnosed by dermatologists with expertise in hair disorders. Sixteen (47%) were diagnosed by dermatologists within a skin-of-color center. Fourteen (41%) initial dermatology consultations were race concordant.

The median patient rating of their dermatologist interactions was excellent (5 on a 5-point scale). Table 2 and Figure 2 show responses to individual items. Respondents saw an average of 3 different providers, both dermatologists and otherwise.

Waiting to See a Dermatologist
Nearly all respondents (31/34 [91%]) recommended that other women with hair loss immediately go see a dermatologist.

Barriers to Care
The top 5 factors reported as most important when initially seeking care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Table 3 shows frequency counts for these freely reported factors.

Quality of Life
The median score on 9 aggregated self-esteem items was 4 on a 5-point scale, representing an agree response to statements such as “I feel embarrassed, self-conscious, or frustrated about my hair loss” (28/34 [82%]) and “My hair loss bothers me” (28/34 [82%])(Table 4). Cronbach α for self-esteem survey items was 0.7826.

For the nonaggregated items, many respondents strongly disagreed with statements pertaining to activities of daily living, including “I take care of where I sit or stand at social gatherings due to my hair loss” (18/34 [53%]), “My hair loss makes it difficult for me to go to the grocery store” (29/34 [85%]), “My hair loss makes it difficult for me to attend faith-based activities” (30/34 [88%]), “My hair loss makes it difficult for me to exercise” (23/34 [68%]), “My hair loss makes it difficult for me to go to work and/or school” (24/34 [71%]), “My hair loss makes it difficult for me to go out with a significant other” (24/34 [71%]), “My hair loss makes it difficult for me to spend time with family” (27/34 [79%]), and “My hair loss makes it difficult for me to go to a hairstylist” (16/34 [47%]).

Comment

The majority of respondents were first to discover their hair loss. Harbingers of CCCA hair loss include paresthesia, tenderness, and itch,⁶ symptoms that are hard to ignore. Unfortunately, many patients notice hair thinning years after the scarring process has begun and a notable amount of hair has already been lost.^6,9

Fifteen percent of respondents learned about their hair loss from their hairstylist. Women of African descent often maintain hairstyles that require frequent interactions with a hair care professional.^7,10 As a result, hairstylists are at the forefront of early alopecia detection and are a valued resource in the black community. Open dialogue between dermatologists and hair care professionals could funnel women with hair loss into treatment before extensive damage.

Fifteen women (44%) recalled a waiting period of several months before seeking medical assistance, and 16 (47%) reported waiting 1 year or more. However, 91% of respondents indicated that women with hair loss should immediately see a physician for evaluation, thus patient experiences underscore the importance of early treatment. In our experience, many patients wait years before presenting to a physician. Some work with their hairstylists first to address the issue, while others do not realize how notable the loss has become. Some have a negative experience with one provider or are told there is nothing that can be done and then wait many years to see a second provider. Proper education of patients, physicians, and hairstylists is important in the identification and prompt treatment of this condition.

It is perhaps to be expected that patients rated interactions with dermatologists as excellent and very good more frequently than interactions with nondermatologists, which may be due to an absence of thorough hair evaluation with nondermatologists. Respondents reported that only half of nondermatologist providers actually examined their scalp during an initial encounter. However, both physician groups had the lowest frequencies of excellent and very good ratings on “understanding of your hair” (Tables 1 and 2). Patients with hair loss seek immediate answers, and often it is the specialist that can give them a firm diagnosis as opposed to a primary care provider. The fact that dermatologists and nondermatologists alike scored poorly on patient-perceived understanding of CCCA indicates an area for improvement within patient-physician interactions and physician knowledge.

The top 5 factors important to respondents when obtaining medical care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Patients with CCCA seeing dermatologists may discern a lack of experience with ethnic hair that leads patients to doubt their physicians’ ability to provide adequate care and decreased shared decision-making.^11,12 These patient perceptions are not unfounded; a 2008 study showed that dermatology residents are not uniformly trained in diseases pertaining to patients with skin of color.¹³ Thus, incorporation of education on skin of color in dermatology training programs is critical.

Finally, hair loss patients often have concerns regarding how medical therapeutics could adversely affect personal hair care regimens, including washing and hairstyling practices. Current research demonstrates that patients consider treatment effectiveness and ability to be integrated into daily routines after establishing medical care.¹⁴ The present study shows that some CCCA patients contemplate how well a therapy will work before seeking medical care, demonstrating that patients continue to have these concerns after establishing medical care. Consideration of treatment effectiveness is important for both patients and providers, as there is minimal evidence behind current CCCA management practices. The ability for treatments to be easily integrated into daily hair care habits is important to maintain patient compliance.

Participants’ median self-esteem scores indicate the effect of CCCA on morale and self-perception. Items scrutinizing this construct had acceptable internal consistency reliability. It is interesting to note that activities of daily living were not impacted by hair loss. Examination of self-esteem is important in the alopecia population because the effect of hair loss on mental status is well documented.^15-17 Low self-esteem has been reported as a prospective risk factor for clinical depression.^18-20 In black patients, clinical depression rates surpass those of Hispanics and non-Hispanic white individuals.²¹ Dermatologists must consider the psychological status of all patients, particularly populations at risk for severe disease.

Limitations of this study include the small (34 participants) and mostly highly educated sample size, limited survey validity, and potential patient bias. Because many patients changed their address and/or telephone number in the time between CCCA diagnosis and the present study, we were left with a small pilot study, which minimizes the impact of our findings. Furthermore, our survey was created by a single expert’s opinion and modeling from preexisting alopecia questionnaires¹⁶; full validity procedures analyzing face, content, and criterion validity were not undertaken. Finally, the majority of respondents were patients of one of the study’s authors (S.S.L.P.), which could influence survey responses. The fact that some providers were hair experts and some were race concordant with their patients also could potentially affect the responses received, which was not analyzed in the present study. Future studies with more respondents from multiple providers would help clarify our preliminary findings.

Conclusion

Analysis of barriers to care and QOL in patients with skin of color is an essential addition to dermatologic discourse. Alopecia is particularly important to investigate, as prior research has found it to be one of the top 5 diagnoses made in patients with skin of color.^3,4 Alopecia has been shown to negatively affect QOL.^15,22,23 This study, although limited by small sample size, suggests CCCA also is a contributor to self-esteem challenges, similar to other forms of hair loss. Patient-physician interactions and personal hairstyling practices are prominent barriers to care for CCCA patients, demonstrating the need for quality education on skin of color and cultural competency in dermatology residencies across the country.

The etiology of central centrifugal cicatricial alopecia (CCCA), a clinical and histological pattern of hair loss on the central scalp, has been well studied. This disease is chronic and progressive, with extensive follicular destruction and eventual burnout.^1,2 Central centrifugal cicatricial alopecia is most commonly seen in patients of African descent and has been shown to be 1 of the 5 most common dermatologic diagnoses in black patients.^3,4 The top 5 dermatologic diagnoses within this population include acne vulgaris (28.4%), dyschromia (19.9%), eczema (9.1%), alopecia (8.3%), and seborrheic dermatitis (6.7%).⁴ The incidence rate of CCCA is estimated to be 5.6%.^3,5 Most patients are women, with onset between the second and fourth decades of life.⁶

Central centrifugal cicatricial alopecia treatment efficacy is inversely correlated with disease duration. The primary goal of treatment is to prevent progression. Efforts are made to stimulate regrowth in areas that are not permanently scarred. When patients present with a substantial amount of scarring hair loss, dermatologists often are limited in their ability to achieve a cosmetically acceptable pattern of growth. Generally, hair is connected to a sense of self-worth in black women, and any type of hair loss has been shown to lead to frustration and decreased self-esteem.⁷ A 1994 study showed that 75% (44/58) of women with androgenetic alopecia had decreased self-esteem and 50% (29/58) had social challenges.⁸

The purpose of this pilot study was to determine the personal, historical, logistical, or environmental factors that preclude women from obtaining medical care for CCCA and to investigate how CCCA affects quality of life (QOL) and psychological well-being.

Methods

The investigators designed a survey study of adult, English-speaking, black women diagnosed with CCCA at the Northwestern University Department of Dermatology (Chicago, Illinois) between 2011 and 2017. Patients were selected from the electronic data warehouse compiled by the Department of Dermatology and were included if they fulfilled the following criteria: evaluated in the dermatology department between September 1, 2011, and September 30, 2017, by any faculty physician; diagnosed with CCCA; and aged 18 years or older. Patients were excluded if they did not speak English, as interpreters were not available. All patients who fulfilled the inclusion criteria provided signed informed consent prior to participation. All surveys were disseminated in the office or via telephone from fall 2016 to spring 2017 and took 10 to 15 minutes to complete. The research was approved by the authors’ institutional review board (IRB ID STU00203449).

Survey Instrument
The CCCA Barriers to Care and Quality of Life Survey is a 53-item survey instrument created by the study’s authors to measure 2 aspects of CCCA hair loss: barriers to medical care and QOL. The initial set of questions pertained to initial hair loss discovery and the number of physicians, both dermatologist and otherwise, seen for hair evaluation. Patients then rated their physician interactions on an ordinal Likert scale (1=poor, 2=fair, 3=good, 4=very good, 5=excellent). If patients saw more than 1 dermatologist or nondermatologist physician, ratings for each provider were included in our analysis. Patients listed the top 3 factors they considered when seeking medical care for their hair loss in order of importance. They also indicated how much they agreed with QOL statements on a Likert scale (1=strongly disagree, 2=disagree, 3=neutral, 4=agree, 5=strongly agree).

Data Analysis
Analyses were completed using data analysis software JMP Pro 13 from SAS and a Microsoft Excel spreadsheet. Continuous data were presented as mean, SD, median, minimum, and maximum. Categorical data were presented as counts and percentages. Nine QOL items were aggregated into a self-esteem category (questions 30–38).

Cronbach α, a statistical measure of internal consistency and how closely related items are in a group, was used to evaluate internal consistency reliability; values of 0.70 or greater indicate acceptable reliability.

Results

Of 501 individuals contacted, 34 completed the survey (7% completion rate). Nonrespondents included 7 who refused to participate and 460 who could not be contacted. All respondents self-identified as black women. Median age at time of survey administration was 46 years (range, 28–79 years); median age at CCCA diagnosis was 42 years (range, 15–73 years). Respondents did not significantly differ in age from nonrespondents (P=.46). The majority of respondents had an associate’s degree, bachelor’s degree, or advanced degree of education (master of arts, doctor of medicine, doctor of jurisprudence, doctor of philosophy); however, 8 women reported completing some college, 1 reported completing high school, and 1 reported no schooling. Three respondents had no health insurance.

Initial Hair Loss Discovery
The majority of respondents (22/34 [65%]) were first to notice their hair loss, while 5 (15%) reported hairstylists as the initial observers. Twelve respondents (35%) initially went to a physician to learn why they were losing hair; 6 (18%) instead utilized hairstylists or the Internet. Fifteen women (44%) waited more than 1 month up to 6 months after noticing hair loss before seeing a physician instead of going immediately within a 4-week period, and 16 (47%) waited 1 year or more.

Nondermatologist Consultation
Almost half (16/34 [47%]) of the women went to a nondermatologist physician regarding their hair loss; of them, half (8/16 [50%]) reported their physician did not examine the scalp, 3 (19%) reported their physician offered a biopsy, and none of them reported that their physician diagnosed them with CCCA. The median patient rating of their nondermatologist physician interactions was good (3 on a 5-point scale). Table 1 and Figure 1 show responses to individual items.

Dermatologist Consultation
All 34 respondents presented to a dermatologist. The majority of respondents (22/34 [65%]) saw either 1 or 2 dermatologists for their hair loss. Three (9%) reported their dermatologist did not examine their scalp. Twelve respondents (35%) reported their dermatologist did not offer a biopsy. Twenty-one respondents (62%) reported a CCCA diagnosis from the first dermatologist they saw. Twenty-three respondents (68%) were diagnosed by dermatologists with expertise in hair disorders. Sixteen (47%) were diagnosed by dermatologists within a skin-of-color center. Fourteen (41%) initial dermatology consultations were race concordant.

The median patient rating of their dermatologist interactions was excellent (5 on a 5-point scale). Table 2 and Figure 2 show responses to individual items. Respondents saw an average of 3 different providers, both dermatologists and otherwise.

Waiting to See a Dermatologist
Nearly all respondents (31/34 [91%]) recommended that other women with hair loss immediately go see a dermatologist.

Barriers to Care
The top 5 factors reported as most important when initially seeking care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Table 3 shows frequency counts for these freely reported factors.

Quality of Life
The median score on 9 aggregated self-esteem items was 4 on a 5-point scale, representing an agree response to statements such as “I feel embarrassed, self-conscious, or frustrated about my hair loss” (28/34 [82%]) and “My hair loss bothers me” (28/34 [82%])(Table 4). Cronbach α for self-esteem survey items was 0.7826.

For the nonaggregated items, many respondents strongly disagreed with statements pertaining to activities of daily living, including “I take care of where I sit or stand at social gatherings due to my hair loss” (18/34 [53%]), “My hair loss makes it difficult for me to go to the grocery store” (29/34 [85%]), “My hair loss makes it difficult for me to attend faith-based activities” (30/34 [88%]), “My hair loss makes it difficult for me to exercise” (23/34 [68%]), “My hair loss makes it difficult for me to go to work and/or school” (24/34 [71%]), “My hair loss makes it difficult for me to go out with a significant other” (24/34 [71%]), “My hair loss makes it difficult for me to spend time with family” (27/34 [79%]), and “My hair loss makes it difficult for me to go to a hairstylist” (16/34 [47%]).

Comment

The majority of respondents were first to discover their hair loss. Harbingers of CCCA hair loss include paresthesia, tenderness, and itch,⁶ symptoms that are hard to ignore. Unfortunately, many patients notice hair thinning years after the scarring process has begun and a notable amount of hair has already been lost.^6,9

Fifteen percent of respondents learned about their hair loss from their hairstylist. Women of African descent often maintain hairstyles that require frequent interactions with a hair care professional.^7,10 As a result, hairstylists are at the forefront of early alopecia detection and are a valued resource in the black community. Open dialogue between dermatologists and hair care professionals could funnel women with hair loss into treatment before extensive damage.

Fifteen women (44%) recalled a waiting period of several months before seeking medical assistance, and 16 (47%) reported waiting 1 year or more. However, 91% of respondents indicated that women with hair loss should immediately see a physician for evaluation, thus patient experiences underscore the importance of early treatment. In our experience, many patients wait years before presenting to a physician. Some work with their hairstylists first to address the issue, while others do not realize how notable the loss has become. Some have a negative experience with one provider or are told there is nothing that can be done and then wait many years to see a second provider. Proper education of patients, physicians, and hairstylists is important in the identification and prompt treatment of this condition.

It is perhaps to be expected that patients rated interactions with dermatologists as excellent and very good more frequently than interactions with nondermatologists, which may be due to an absence of thorough hair evaluation with nondermatologists. Respondents reported that only half of nondermatologist providers actually examined their scalp during an initial encounter. However, both physician groups had the lowest frequencies of excellent and very good ratings on “understanding of your hair” (Tables 1 and 2). Patients with hair loss seek immediate answers, and often it is the specialist that can give them a firm diagnosis as opposed to a primary care provider. The fact that dermatologists and nondermatologists alike scored poorly on patient-perceived understanding of CCCA indicates an area for improvement within patient-physician interactions and physician knowledge.

The top 5 factors important to respondents when obtaining medical care included the physician’s experience with black hair and CCCA, the patient’s personal hairstyling practices, the physician’s ethnicity, availability of effective treatment options, and treatment cost. Patients with CCCA seeing dermatologists may discern a lack of experience with ethnic hair that leads patients to doubt their physicians’ ability to provide adequate care and decreased shared decision-making.^11,12 These patient perceptions are not unfounded; a 2008 study showed that dermatology residents are not uniformly trained in diseases pertaining to patients with skin of color.¹³ Thus, incorporation of education on skin of color in dermatology training programs is critical.

Finally, hair loss patients often have concerns regarding how medical therapeutics could adversely affect personal hair care regimens, including washing and hairstyling practices. Current research demonstrates that patients consider treatment effectiveness and ability to be integrated into daily routines after establishing medical care.¹⁴ The present study shows that some CCCA patients contemplate how well a therapy will work before seeking medical care, demonstrating that patients continue to have these concerns after establishing medical care. Consideration of treatment effectiveness is important for both patients and providers, as there is minimal evidence behind current CCCA management practices. The ability for treatments to be easily integrated into daily hair care habits is important to maintain patient compliance.

Participants’ median self-esteem scores indicate the effect of CCCA on morale and self-perception. Items scrutinizing this construct had acceptable internal consistency reliability. It is interesting to note that activities of daily living were not impacted by hair loss. Examination of self-esteem is important in the alopecia population because the effect of hair loss on mental status is well documented.^15-17 Low self-esteem has been reported as a prospective risk factor for clinical depression.^18-20 In black patients, clinical depression rates surpass those of Hispanics and non-Hispanic white individuals.²¹ Dermatologists must consider the psychological status of all patients, particularly populations at risk for severe disease.

Limitations of this study include the small (34 participants) and mostly highly educated sample size, limited survey validity, and potential patient bias. Because many patients changed their address and/or telephone number in the time between CCCA diagnosis and the present study, we were left with a small pilot study, which minimizes the impact of our findings. Furthermore, our survey was created by a single expert’s opinion and modeling from preexisting alopecia questionnaires¹⁶; full validity procedures analyzing face, content, and criterion validity were not undertaken. Finally, the majority of respondents were patients of one of the study’s authors (S.S.L.P.), which could influence survey responses. The fact that some providers were hair experts and some were race concordant with their patients also could potentially affect the responses received, which was not analyzed in the present study. Future studies with more respondents from multiple providers would help clarify our preliminary findings.

Conclusion

Analysis of barriers to care and QOL in patients with skin of color is an essential addition to dermatologic discourse. Alopecia is particularly important to investigate, as prior research has found it to be one of the top 5 diagnoses made in patients with skin of color.^3,4 Alopecia has been shown to negatively affect QOL.^15,22,23 This study, although limited by small sample size, suggests CCCA also is a contributor to self-esteem challenges, similar to other forms of hair loss. Patient-physician interactions and personal hairstyling practices are prominent barriers to care for CCCA patients, demonstrating the need for quality education on skin of color and cultural competency in dermatology residencies across the country.