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Scalp Psoriasis: Weighing Treatment Options
Scalp psoriasis often is the initial presentation of psoriasis, and it can be one of the most challenging aspects of the disease. It can be difficult to treat for several reasons. First, hair can interfere with topical therapy reaching its site of action on the scalp. Second, facial skin also can be exposed to these treatments with the associated risk for adverse events. Finally, compliance often is difficult.
An evidence-based review published online on September 21 in the American Journal of Clinical Dermatology examined treatments for scalp psoriasis, including newer systemic therapies. Of 475 studies initially identified from PubMed and 845 from Embase (up to May 2016), the review included 27 clinical trials, 4 papers reporting pooled analyses of other clinical trials, 10 open-label trials, 1 case series, and 2 case reports after excluding non-English literature.
Wang and Tsai noted that few randomized controlled trials have been performed specifically in scalp psoriasis. The authors found that topical corticosteroids provide good effects and are usually recommended as first-line treatment. Calcipotriol–betamethasone dipropionate is more highly effective than either of its individual components.
The analysis also suggested that localized phototherapy is better than generalized phototherapy on hair-bearing areas. Methotrexate, cyclosporine, fumaric acid esters, and acitretin are well-recognized agents in the treatment of psoriasis, but they located no published randomized controlled trials specifically evaluating these agents in scalp psoriasis. Wang and Tsai also commented that biologics and new small-molecule agents show excellent effects on scalp psoriasis, but the high cost of these treatments mean they may be limited to use in extensive scalp psoriasis. They suggested that more controlled studies are needed for an evidence-based approach to scalp psoriasis.
What’s the issue?
Scalp psoriasis can be an isolated condition or may occur in association with more extensive disease. There has been increased attention to its treatment over the last several years, with several new options. What is your preferred approach to scalp psoriasis?
Scalp psoriasis often is the initial presentation of psoriasis, and it can be one of the most challenging aspects of the disease. It can be difficult to treat for several reasons. First, hair can interfere with topical therapy reaching its site of action on the scalp. Second, facial skin also can be exposed to these treatments with the associated risk for adverse events. Finally, compliance often is difficult.
An evidence-based review published online on September 21 in the American Journal of Clinical Dermatology examined treatments for scalp psoriasis, including newer systemic therapies. Of 475 studies initially identified from PubMed and 845 from Embase (up to May 2016), the review included 27 clinical trials, 4 papers reporting pooled analyses of other clinical trials, 10 open-label trials, 1 case series, and 2 case reports after excluding non-English literature.
Wang and Tsai noted that few randomized controlled trials have been performed specifically in scalp psoriasis. The authors found that topical corticosteroids provide good effects and are usually recommended as first-line treatment. Calcipotriol–betamethasone dipropionate is more highly effective than either of its individual components.
The analysis also suggested that localized phototherapy is better than generalized phototherapy on hair-bearing areas. Methotrexate, cyclosporine, fumaric acid esters, and acitretin are well-recognized agents in the treatment of psoriasis, but they located no published randomized controlled trials specifically evaluating these agents in scalp psoriasis. Wang and Tsai also commented that biologics and new small-molecule agents show excellent effects on scalp psoriasis, but the high cost of these treatments mean they may be limited to use in extensive scalp psoriasis. They suggested that more controlled studies are needed for an evidence-based approach to scalp psoriasis.
What’s the issue?
Scalp psoriasis can be an isolated condition or may occur in association with more extensive disease. There has been increased attention to its treatment over the last several years, with several new options. What is your preferred approach to scalp psoriasis?
Scalp psoriasis often is the initial presentation of psoriasis, and it can be one of the most challenging aspects of the disease. It can be difficult to treat for several reasons. First, hair can interfere with topical therapy reaching its site of action on the scalp. Second, facial skin also can be exposed to these treatments with the associated risk for adverse events. Finally, compliance often is difficult.
An evidence-based review published online on September 21 in the American Journal of Clinical Dermatology examined treatments for scalp psoriasis, including newer systemic therapies. Of 475 studies initially identified from PubMed and 845 from Embase (up to May 2016), the review included 27 clinical trials, 4 papers reporting pooled analyses of other clinical trials, 10 open-label trials, 1 case series, and 2 case reports after excluding non-English literature.
Wang and Tsai noted that few randomized controlled trials have been performed specifically in scalp psoriasis. The authors found that topical corticosteroids provide good effects and are usually recommended as first-line treatment. Calcipotriol–betamethasone dipropionate is more highly effective than either of its individual components.
The analysis also suggested that localized phototherapy is better than generalized phototherapy on hair-bearing areas. Methotrexate, cyclosporine, fumaric acid esters, and acitretin are well-recognized agents in the treatment of psoriasis, but they located no published randomized controlled trials specifically evaluating these agents in scalp psoriasis. Wang and Tsai also commented that biologics and new small-molecule agents show excellent effects on scalp psoriasis, but the high cost of these treatments mean they may be limited to use in extensive scalp psoriasis. They suggested that more controlled studies are needed for an evidence-based approach to scalp psoriasis.
What’s the issue?
Scalp psoriasis can be an isolated condition or may occur in association with more extensive disease. There has been increased attention to its treatment over the last several years, with several new options. What is your preferred approach to scalp psoriasis?
Biopsy scalp area for alopecia diagnosis
BOSTON – Getting a proper scalp biopsy and providing the dermatopathologist with a good supporting history are important elements in diagnosing a patient with hair loss, according to Eleanor Knopp, MD, a dermatologist and dermatopathologist with Group Health Permanente, Seattle.
The keys to a good scalp biopsy in a patient with alopecia are to take an adequate sample of scalp in both size and degree of involvement. With regard to where to biopsy, “it’s important ... to select an area of advanced thinning if you’re doing a biopsy of a nonscarring alopecia,” Dr. Knopp said at the American Academy of Dermatology summer meeting. She advised being “generous” with an anesthetic, preferably one containing epinephrine, to help keep the wound dry and to help with visualization during the procedure.
Evaluating for the presence or absence of follicular ostia with a dermatoscope helps distinguish scarring from nonscarring alopecia. Scarring alopecias typically show loss of follicular ostia, she noted.
While this method is effective at identifying nonscarring areas in white patients, it can be difficult to appreciate the disappearance of follicular ostia with a dermatoscope in patients of African descent or patients with darkly pigmented skin. In these patients, eccrine ostia appear as white pinpoint dots under a dermatoscope and mimic the appearance of follicular ostia, despite the presence of scarring alopecia, she noted.
In this situation, Dr. Knopp said the threshold for biopsying patients with darkly pigmented skin should be lower to rule out an early cicatricial alopecia.
For any specimen sent to the dermatopathologist, it is important to note patient characteristics, including age and race, duration of the condition, and clinical pattern. Not only is race helpful for interpreting what is seen in the specimen, but certain racial groups have higher predilections for certain diseases. There are also differences in normal hair densities depending on race although there can be a wide range even within a race, she added. Providing a photo of the involved area of the scalp is also a good idea, she added.
When biopsying a scarring alopecia, Dr. Knopp said that her preference is to find an area of relatively early thinning with visible erythema, and scale if it is present, “so that you know you have active inflammatory disease, but it’s not so advanced that you’re just seeing end-stage changes of scarring.”
It is worth having a discussion with the dermatopathologist about sectioning specimens, Dr. Knopp said. The consensus among most dermatopathologists is that horizontal sections are “absolutely the way to go for nonscarring alopecias,” but some dermatopathologists strongly prefer vertical sections, especially in cicatricial alopecias. Clinicians can always choose among the many reference laboratories to obtain the type of sections they prefer.
In cases of cicatricial alopecia, Dr. Knopp cautioned that clinicians may see “juicy pustules or fluctuant nodules” and consider these findings indicative of a highly active area of disease, but these changes may obscure early findings that are helpful to a pathologist. A better choice for a biopsy site is an area of early involvement that is not too inflamed and not so advanced that it is just scar, she noted.
Another potential pitfall is the temptation to biopsy a tuft of hairs. If there is polytrichia or compounding of follicles, it may be tempting to fit the punch tool over what are also sometimes called “doll’s hairs.” But those structures are nonspecific, end-stage features of many different cicatricial alopecias, including lichen planopilaris, central centrifugal cicatricial alopecia, and even lupus. Instead, Dr. Knopp recommended taking a specimen at the periphery where compounding is not present but where there is thinning and active inflammation.
Dr. Knopp, also with the University of Washington, Seattle, reported no financial relationships.
BOSTON – Getting a proper scalp biopsy and providing the dermatopathologist with a good supporting history are important elements in diagnosing a patient with hair loss, according to Eleanor Knopp, MD, a dermatologist and dermatopathologist with Group Health Permanente, Seattle.
The keys to a good scalp biopsy in a patient with alopecia are to take an adequate sample of scalp in both size and degree of involvement. With regard to where to biopsy, “it’s important ... to select an area of advanced thinning if you’re doing a biopsy of a nonscarring alopecia,” Dr. Knopp said at the American Academy of Dermatology summer meeting. She advised being “generous” with an anesthetic, preferably one containing epinephrine, to help keep the wound dry and to help with visualization during the procedure.
Evaluating for the presence or absence of follicular ostia with a dermatoscope helps distinguish scarring from nonscarring alopecia. Scarring alopecias typically show loss of follicular ostia, she noted.
While this method is effective at identifying nonscarring areas in white patients, it can be difficult to appreciate the disappearance of follicular ostia with a dermatoscope in patients of African descent or patients with darkly pigmented skin. In these patients, eccrine ostia appear as white pinpoint dots under a dermatoscope and mimic the appearance of follicular ostia, despite the presence of scarring alopecia, she noted.
In this situation, Dr. Knopp said the threshold for biopsying patients with darkly pigmented skin should be lower to rule out an early cicatricial alopecia.
For any specimen sent to the dermatopathologist, it is important to note patient characteristics, including age and race, duration of the condition, and clinical pattern. Not only is race helpful for interpreting what is seen in the specimen, but certain racial groups have higher predilections for certain diseases. There are also differences in normal hair densities depending on race although there can be a wide range even within a race, she added. Providing a photo of the involved area of the scalp is also a good idea, she added.
When biopsying a scarring alopecia, Dr. Knopp said that her preference is to find an area of relatively early thinning with visible erythema, and scale if it is present, “so that you know you have active inflammatory disease, but it’s not so advanced that you’re just seeing end-stage changes of scarring.”
It is worth having a discussion with the dermatopathologist about sectioning specimens, Dr. Knopp said. The consensus among most dermatopathologists is that horizontal sections are “absolutely the way to go for nonscarring alopecias,” but some dermatopathologists strongly prefer vertical sections, especially in cicatricial alopecias. Clinicians can always choose among the many reference laboratories to obtain the type of sections they prefer.
In cases of cicatricial alopecia, Dr. Knopp cautioned that clinicians may see “juicy pustules or fluctuant nodules” and consider these findings indicative of a highly active area of disease, but these changes may obscure early findings that are helpful to a pathologist. A better choice for a biopsy site is an area of early involvement that is not too inflamed and not so advanced that it is just scar, she noted.
Another potential pitfall is the temptation to biopsy a tuft of hairs. If there is polytrichia or compounding of follicles, it may be tempting to fit the punch tool over what are also sometimes called “doll’s hairs.” But those structures are nonspecific, end-stage features of many different cicatricial alopecias, including lichen planopilaris, central centrifugal cicatricial alopecia, and even lupus. Instead, Dr. Knopp recommended taking a specimen at the periphery where compounding is not present but where there is thinning and active inflammation.
Dr. Knopp, also with the University of Washington, Seattle, reported no financial relationships.
BOSTON – Getting a proper scalp biopsy and providing the dermatopathologist with a good supporting history are important elements in diagnosing a patient with hair loss, according to Eleanor Knopp, MD, a dermatologist and dermatopathologist with Group Health Permanente, Seattle.
The keys to a good scalp biopsy in a patient with alopecia are to take an adequate sample of scalp in both size and degree of involvement. With regard to where to biopsy, “it’s important ... to select an area of advanced thinning if you’re doing a biopsy of a nonscarring alopecia,” Dr. Knopp said at the American Academy of Dermatology summer meeting. She advised being “generous” with an anesthetic, preferably one containing epinephrine, to help keep the wound dry and to help with visualization during the procedure.
Evaluating for the presence or absence of follicular ostia with a dermatoscope helps distinguish scarring from nonscarring alopecia. Scarring alopecias typically show loss of follicular ostia, she noted.
While this method is effective at identifying nonscarring areas in white patients, it can be difficult to appreciate the disappearance of follicular ostia with a dermatoscope in patients of African descent or patients with darkly pigmented skin. In these patients, eccrine ostia appear as white pinpoint dots under a dermatoscope and mimic the appearance of follicular ostia, despite the presence of scarring alopecia, she noted.
In this situation, Dr. Knopp said the threshold for biopsying patients with darkly pigmented skin should be lower to rule out an early cicatricial alopecia.
For any specimen sent to the dermatopathologist, it is important to note patient characteristics, including age and race, duration of the condition, and clinical pattern. Not only is race helpful for interpreting what is seen in the specimen, but certain racial groups have higher predilections for certain diseases. There are also differences in normal hair densities depending on race although there can be a wide range even within a race, she added. Providing a photo of the involved area of the scalp is also a good idea, she added.
When biopsying a scarring alopecia, Dr. Knopp said that her preference is to find an area of relatively early thinning with visible erythema, and scale if it is present, “so that you know you have active inflammatory disease, but it’s not so advanced that you’re just seeing end-stage changes of scarring.”
It is worth having a discussion with the dermatopathologist about sectioning specimens, Dr. Knopp said. The consensus among most dermatopathologists is that horizontal sections are “absolutely the way to go for nonscarring alopecias,” but some dermatopathologists strongly prefer vertical sections, especially in cicatricial alopecias. Clinicians can always choose among the many reference laboratories to obtain the type of sections they prefer.
In cases of cicatricial alopecia, Dr. Knopp cautioned that clinicians may see “juicy pustules or fluctuant nodules” and consider these findings indicative of a highly active area of disease, but these changes may obscure early findings that are helpful to a pathologist. A better choice for a biopsy site is an area of early involvement that is not too inflamed and not so advanced that it is just scar, she noted.
Another potential pitfall is the temptation to biopsy a tuft of hairs. If there is polytrichia or compounding of follicles, it may be tempting to fit the punch tool over what are also sometimes called “doll’s hairs.” But those structures are nonspecific, end-stage features of many different cicatricial alopecias, including lichen planopilaris, central centrifugal cicatricial alopecia, and even lupus. Instead, Dr. Knopp recommended taking a specimen at the periphery where compounding is not present but where there is thinning and active inflammation.
Dr. Knopp, also with the University of Washington, Seattle, reported no financial relationships.
Cosmetic Corner: Dermatologists Weigh in on Nail Care Products
To improve patient care and outcomes, leading dermatologists offered their recommendations on nail care products. Consideration must be given to:
- Aquaphor Healing Ointment
Beiersdorf, Inc
Recommended by Gary Goldenberg, MD, New York, New York
- Biotin Oral Supplements
Manufacturers vary
“Biotin is a helpful supplement for brittle nails. It may take 6 months to see improvement in the nails.”—Shari Lipner, MD, PhD, New York, New York
Recommended by Gary Goldenberg, MD, New York, New York
- Deep Comfort Hand and Cuticle Cream
Clinique
“It has good hydration for cuticles with sodium hyaluronate and squalene. It also is fragrance free.”—Anthony M. Rossi, MD, New York, New York
- Genadur
Medimetriks Pharmaceuticals, Inc
Recommended by Gary Goldenberg, MD, New York, New York
- Lanolin-Rich Nail Conditioner
Elon
“It’s great for moisturizing and nail hardening.”—Marta Rendon, MD, Boca Raton, Florida
- Nail Renewal System
Dr. Dana
“Developed by dermatologist Dr. Dana Stern, the system combines glycolic acid to improve discoloration and ridging, along with hydrating and strengthening botanicals to improve the look, feel, and overall health of the nails.”— Joshua Zeichner, MD, New York, New York
Cutis invites readers to send us their recommendations. Acne scar treatments, self-tanners, and cleansing devices will be featured in upcoming editions of Cosmetic Corner. Please e-mail your recommendation(s) to the Editorial Office.
Disclaimer: Opinions expressed herein do not necessarily reflect those of Cutis or Frontline Medical Communications Inc. and shall not be used for product endorsement purposes. Any reference made to a specific commercial product does not indicate or imply that Cutis or Frontline Medical Communications Inc. endorses, recommends, or favors the product mentioned. No guarantee is given to the effects of recommended products.
To improve patient care and outcomes, leading dermatologists offered their recommendations on nail care products. Consideration must be given to:
- Aquaphor Healing Ointment
Beiersdorf, Inc
Recommended by Gary Goldenberg, MD, New York, New York
- Biotin Oral Supplements
Manufacturers vary
“Biotin is a helpful supplement for brittle nails. It may take 6 months to see improvement in the nails.”—Shari Lipner, MD, PhD, New York, New York
Recommended by Gary Goldenberg, MD, New York, New York
- Deep Comfort Hand and Cuticle Cream
Clinique
“It has good hydration for cuticles with sodium hyaluronate and squalene. It also is fragrance free.”—Anthony M. Rossi, MD, New York, New York
- Genadur
Medimetriks Pharmaceuticals, Inc
Recommended by Gary Goldenberg, MD, New York, New York
- Lanolin-Rich Nail Conditioner
Elon
“It’s great for moisturizing and nail hardening.”—Marta Rendon, MD, Boca Raton, Florida
- Nail Renewal System
Dr. Dana
“Developed by dermatologist Dr. Dana Stern, the system combines glycolic acid to improve discoloration and ridging, along with hydrating and strengthening botanicals to improve the look, feel, and overall health of the nails.”— Joshua Zeichner, MD, New York, New York
Cutis invites readers to send us their recommendations. Acne scar treatments, self-tanners, and cleansing devices will be featured in upcoming editions of Cosmetic Corner. Please e-mail your recommendation(s) to the Editorial Office.
Disclaimer: Opinions expressed herein do not necessarily reflect those of Cutis or Frontline Medical Communications Inc. and shall not be used for product endorsement purposes. Any reference made to a specific commercial product does not indicate or imply that Cutis or Frontline Medical Communications Inc. endorses, recommends, or favors the product mentioned. No guarantee is given to the effects of recommended products.
To improve patient care and outcomes, leading dermatologists offered their recommendations on nail care products. Consideration must be given to:
- Aquaphor Healing Ointment
Beiersdorf, Inc
Recommended by Gary Goldenberg, MD, New York, New York
- Biotin Oral Supplements
Manufacturers vary
“Biotin is a helpful supplement for brittle nails. It may take 6 months to see improvement in the nails.”—Shari Lipner, MD, PhD, New York, New York
Recommended by Gary Goldenberg, MD, New York, New York
- Deep Comfort Hand and Cuticle Cream
Clinique
“It has good hydration for cuticles with sodium hyaluronate and squalene. It also is fragrance free.”—Anthony M. Rossi, MD, New York, New York
- Genadur
Medimetriks Pharmaceuticals, Inc
Recommended by Gary Goldenberg, MD, New York, New York
- Lanolin-Rich Nail Conditioner
Elon
“It’s great for moisturizing and nail hardening.”—Marta Rendon, MD, Boca Raton, Florida
- Nail Renewal System
Dr. Dana
“Developed by dermatologist Dr. Dana Stern, the system combines glycolic acid to improve discoloration and ridging, along with hydrating and strengthening botanicals to improve the look, feel, and overall health of the nails.”— Joshua Zeichner, MD, New York, New York
Cutis invites readers to send us their recommendations. Acne scar treatments, self-tanners, and cleansing devices will be featured in upcoming editions of Cosmetic Corner. Please e-mail your recommendation(s) to the Editorial Office.
Disclaimer: Opinions expressed herein do not necessarily reflect those of Cutis or Frontline Medical Communications Inc. and shall not be used for product endorsement purposes. Any reference made to a specific commercial product does not indicate or imply that Cutis or Frontline Medical Communications Inc. endorses, recommends, or favors the product mentioned. No guarantee is given to the effects of recommended products.
An Unusual Case of Folliculitis Spinulosa Decalvans
Case Report
A 24-year-old man was referred to the dermatology department for evaluation of pustules, atrophic scars, and alopecia on the scalp of 6 years’ duration. Six years prior, erythema, scaling, and follicular keratotic papules had appeared on the superciliary arches, and he started to lose hair from the eyebrows. Three months later, he developed mildly pruritic and painful scaling and pustules on the scalp. These lesions resolved with atrophic scarring accompanied by alopecia. One year later, follicular keratotic papules developed on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae. Two years later, direct microscopy of the lesions on the scalp and fungal culture were negative. After 2 weeks of treatment with roxithromycin (0.15 g twice daily), the scalp pustules dried out and resolved; however, they recurred when the patient stopped taking the medication. Six months later, he was started on isotretinoin treatment (10 mg once daily) for half a year, but no improvement was seen. His parents were nonconsanguineous, and no other family members were affected.
Dermatologic examination revealed large areas of atrophic scarring and alopecia on the scalp. Only a few solitary hairs remained on the top of the head, with the follicles surrounded by keratotic papules, pustules, and black scabs. There was sparse hair on the forehead and temples and scattered hair clusters in the occipital region near the hairline. These follicles also were associated with keratotic papules (Figure 1A). Erythema, scales, and follicular keratotic papules of the superciliary arches with sparse eyebrows and axillary hairs were noted. Follicular keratotic papules also were observed on the cheeks, axillae, chest, abdomen, back, lateral upper arms, and thighs. Dental examination revealed a large space between the upper anterior teeth and the lower anterior teeth. The upper anterior teeth were anteverted, there was congenital absence of right lower central incisors, and the anterior teeth were in deep overbite and overjet (Figure 1B). There was gingival atrophy and calculus dentalis in the upper and lower teeth. He had a fissured tongue with atrophic filiform papillae (Figure 1C).
Laboratory testing of the blood, urine, stool, hepatic and renal function, and serum vitamin B2 and B12 levelswere all within reference range. A panoramic radiograph of the occlusal surface showed congenital absence of right lower central incisors (Figure 2), and a lateral projection of a cranial radiograph confirmed that the anterior teeth were in deep overbite and overjet. Direct microscopy and fungal culture of material collected from the dorsal tongue were negative. Direct microscopy and fungal culture of diseased hairs also were negative. A rapid plasma reagin test, Treponema pallidum hemagglutination assay, and human immunodeficiency virus test were negative. Staphylococcus aureus was isolated from the scalp pustules, and in vitro drug susceptibility testing showed that it was sensitive to clarithromycin and moxifloxacin. Pathological examination of a biopsy of the occipital skin lesions showed a thickened epidermal spinous layer and massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles (Figure 3). Pathological examination of the skin lesions on the superciliary arch also showed infiltration of inflammatory cells in the dermis around the hair follicles.
Based on these findings, a diagnosis of folliculitis spinulosa decalvans (FSD) was made and the patient was started on clarithromycin (0.25 g twice daily), metronidazole (0.2 g 3 times daily), viaminate (50 mg 3 times daily), and fusidic acid cream (coating the affected area twice daily). When he returned for follow-up 1 month later, the pustules had disappeared and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.
Comment
Folliculitis spinulosa decalvans, along with keratosis follicularis spinulosa decalvans (KFSD), keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum, belongs to a group of diseases that includes keratosis pilaris atrophicans. In 1994, Oranje et al1 suggested the term folliculitis spinulosa decalvans, with signs including persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp, which may be exacerbated at puberty. Staphylococcus aureus was isolated from the pustules in one study2; however, in another study, repeated cultures were negative.3 Although the main inheritance pattern of KFSD is X-linked, autosomal-dominant inheritance is more common in FSD. Furthermore, there are certain differences in the clinical manifestations of these 2 conditions. Therefore, it remains controversial if FSD is an independent disease or merely a subtype of KFSD.
Our patient’s symptoms manifested after puberty, primarily pustules as well as atrophic and scarring alopecia of the scalp and follicular keratotic papules on the head, face, trunk, lateral upper arms, and thighs. Pathologic examination showed massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles. The clinical and histopathologic findings met the diagnostic criteria for FSD.
Folliculitis spinulosa decalvans is a rare clinical condition with few cases reported.3-5 In addition to the aforementioned characteristic clinical manifestations, our patient also had dental anomalies, a fissured tongue, and atrophy of the tongue papillae, which are not known to be associated with FSD. Dental anomalies are characteristic of patients with Down syndrome, ectodermal dysplasia, Papillon-Lefèvre syndrome, and other conditions.6 Fissured tongue is a normal variant that occurs in 5% to 11% of individuals. It also is a classic but nonspecific feature of Melkersson-Rosenthal syndrome and may occur in psoriasis, Down syndrome, acromegaly, and Sjögren syndrome.7 Atrophy of the tongue papillae is associated with anemia, pellagra, Sjögren syndrome, candidiasis, and other conditions.8 Because there are no known reports of associations between FSD and any of these oral manifestations, it is possible that they were unrelated to FSD in our patient.
Folliculitis spinulosa decalvans usually is recurrent and there is no consistently effective treatment for it. Kunte et al4 reported that dapsone (100 mg/d) led to resolution of scalp inflammation and pustules within 1 month. Romine et al2 reported that a 3-week course of dichloroxacillin (250 mg 4 times daily) induced disappearance of pustules around the hair follicles. However, Hallai et al5 reported a patient who was resistant to isotretinoin treatment. In our case, after 1 month of treatment with clarithromycin, metronidazole, viaminate, and fusidic acid cream, the pustules had resolved and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.
Conclusion
We report a case of FSD with dental anomalies, a fissured tongue, and atrophy of tongue papillae, none of which have previously been reported in association with FSD. We, therefore, believe that our patient’s oral manifestations are unrelated to FSD.
- Oranje AP, van Osch LD, Oosterwijk JC. Keratosis pilaris atrophicans. one heterogeneous disease or a symptom in different clinical entities? Arch Dermatol. 1994;13:500-502.
- Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris. keratosis follicularis spinulosa decalvans. Arch Dermatol. 1997;13:381-384.
- Di Lernia V, Ricci C. Folliculitis spinulosa decalvans: an uncommon entity within the keratosis pilaris atrophicans spectrum. Pediatr Dermatol. 2006;23:255-258.
- Kunte C, Loeser C, Wolff H. Folliculitis spinulosa decalvans: successful therapy with dapsone. J Am Acad Dermatol. 1998;39(5, pt 2):891-892.
- Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
- Scully C, Hegarty A. The oral cavity and lips. In: Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. 8th ed. Oxford, England: Wiley-Blackwell; 2010:69.7-69.10.
- Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Companies; 2007:643.
- Mulliken RA, Casner MJ. Oral manifestations of systemic disease. Emerg Med Clin North Am. 2000;18:565-575.
Case Report
A 24-year-old man was referred to the dermatology department for evaluation of pustules, atrophic scars, and alopecia on the scalp of 6 years’ duration. Six years prior, erythema, scaling, and follicular keratotic papules had appeared on the superciliary arches, and he started to lose hair from the eyebrows. Three months later, he developed mildly pruritic and painful scaling and pustules on the scalp. These lesions resolved with atrophic scarring accompanied by alopecia. One year later, follicular keratotic papules developed on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae. Two years later, direct microscopy of the lesions on the scalp and fungal culture were negative. After 2 weeks of treatment with roxithromycin (0.15 g twice daily), the scalp pustules dried out and resolved; however, they recurred when the patient stopped taking the medication. Six months later, he was started on isotretinoin treatment (10 mg once daily) for half a year, but no improvement was seen. His parents were nonconsanguineous, and no other family members were affected.
Dermatologic examination revealed large areas of atrophic scarring and alopecia on the scalp. Only a few solitary hairs remained on the top of the head, with the follicles surrounded by keratotic papules, pustules, and black scabs. There was sparse hair on the forehead and temples and scattered hair clusters in the occipital region near the hairline. These follicles also were associated with keratotic papules (Figure 1A). Erythema, scales, and follicular keratotic papules of the superciliary arches with sparse eyebrows and axillary hairs were noted. Follicular keratotic papules also were observed on the cheeks, axillae, chest, abdomen, back, lateral upper arms, and thighs. Dental examination revealed a large space between the upper anterior teeth and the lower anterior teeth. The upper anterior teeth were anteverted, there was congenital absence of right lower central incisors, and the anterior teeth were in deep overbite and overjet (Figure 1B). There was gingival atrophy and calculus dentalis in the upper and lower teeth. He had a fissured tongue with atrophic filiform papillae (Figure 1C).
Laboratory testing of the blood, urine, stool, hepatic and renal function, and serum vitamin B2 and B12 levelswere all within reference range. A panoramic radiograph of the occlusal surface showed congenital absence of right lower central incisors (Figure 2), and a lateral projection of a cranial radiograph confirmed that the anterior teeth were in deep overbite and overjet. Direct microscopy and fungal culture of material collected from the dorsal tongue were negative. Direct microscopy and fungal culture of diseased hairs also were negative. A rapid plasma reagin test, Treponema pallidum hemagglutination assay, and human immunodeficiency virus test were negative. Staphylococcus aureus was isolated from the scalp pustules, and in vitro drug susceptibility testing showed that it was sensitive to clarithromycin and moxifloxacin. Pathological examination of a biopsy of the occipital skin lesions showed a thickened epidermal spinous layer and massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles (Figure 3). Pathological examination of the skin lesions on the superciliary arch also showed infiltration of inflammatory cells in the dermis around the hair follicles.
Based on these findings, a diagnosis of folliculitis spinulosa decalvans (FSD) was made and the patient was started on clarithromycin (0.25 g twice daily), metronidazole (0.2 g 3 times daily), viaminate (50 mg 3 times daily), and fusidic acid cream (coating the affected area twice daily). When he returned for follow-up 1 month later, the pustules had disappeared and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.
Comment
Folliculitis spinulosa decalvans, along with keratosis follicularis spinulosa decalvans (KFSD), keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum, belongs to a group of diseases that includes keratosis pilaris atrophicans. In 1994, Oranje et al1 suggested the term folliculitis spinulosa decalvans, with signs including persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp, which may be exacerbated at puberty. Staphylococcus aureus was isolated from the pustules in one study2; however, in another study, repeated cultures were negative.3 Although the main inheritance pattern of KFSD is X-linked, autosomal-dominant inheritance is more common in FSD. Furthermore, there are certain differences in the clinical manifestations of these 2 conditions. Therefore, it remains controversial if FSD is an independent disease or merely a subtype of KFSD.
Our patient’s symptoms manifested after puberty, primarily pustules as well as atrophic and scarring alopecia of the scalp and follicular keratotic papules on the head, face, trunk, lateral upper arms, and thighs. Pathologic examination showed massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles. The clinical and histopathologic findings met the diagnostic criteria for FSD.
Folliculitis spinulosa decalvans is a rare clinical condition with few cases reported.3-5 In addition to the aforementioned characteristic clinical manifestations, our patient also had dental anomalies, a fissured tongue, and atrophy of the tongue papillae, which are not known to be associated with FSD. Dental anomalies are characteristic of patients with Down syndrome, ectodermal dysplasia, Papillon-Lefèvre syndrome, and other conditions.6 Fissured tongue is a normal variant that occurs in 5% to 11% of individuals. It also is a classic but nonspecific feature of Melkersson-Rosenthal syndrome and may occur in psoriasis, Down syndrome, acromegaly, and Sjögren syndrome.7 Atrophy of the tongue papillae is associated with anemia, pellagra, Sjögren syndrome, candidiasis, and other conditions.8 Because there are no known reports of associations between FSD and any of these oral manifestations, it is possible that they were unrelated to FSD in our patient.
Folliculitis spinulosa decalvans usually is recurrent and there is no consistently effective treatment for it. Kunte et al4 reported that dapsone (100 mg/d) led to resolution of scalp inflammation and pustules within 1 month. Romine et al2 reported that a 3-week course of dichloroxacillin (250 mg 4 times daily) induced disappearance of pustules around the hair follicles. However, Hallai et al5 reported a patient who was resistant to isotretinoin treatment. In our case, after 1 month of treatment with clarithromycin, metronidazole, viaminate, and fusidic acid cream, the pustules had resolved and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.
Conclusion
We report a case of FSD with dental anomalies, a fissured tongue, and atrophy of tongue papillae, none of which have previously been reported in association with FSD. We, therefore, believe that our patient’s oral manifestations are unrelated to FSD.
Case Report
A 24-year-old man was referred to the dermatology department for evaluation of pustules, atrophic scars, and alopecia on the scalp of 6 years’ duration. Six years prior, erythema, scaling, and follicular keratotic papules had appeared on the superciliary arches, and he started to lose hair from the eyebrows. Three months later, he developed mildly pruritic and painful scaling and pustules on the scalp. These lesions resolved with atrophic scarring accompanied by alopecia. One year later, follicular keratotic papules developed on the cheeks, chest, abdomen, back, lateral upper arms, thighs, and axillae. Two years later, direct microscopy of the lesions on the scalp and fungal culture were negative. After 2 weeks of treatment with roxithromycin (0.15 g twice daily), the scalp pustules dried out and resolved; however, they recurred when the patient stopped taking the medication. Six months later, he was started on isotretinoin treatment (10 mg once daily) for half a year, but no improvement was seen. His parents were nonconsanguineous, and no other family members were affected.
Dermatologic examination revealed large areas of atrophic scarring and alopecia on the scalp. Only a few solitary hairs remained on the top of the head, with the follicles surrounded by keratotic papules, pustules, and black scabs. There was sparse hair on the forehead and temples and scattered hair clusters in the occipital region near the hairline. These follicles also were associated with keratotic papules (Figure 1A). Erythema, scales, and follicular keratotic papules of the superciliary arches with sparse eyebrows and axillary hairs were noted. Follicular keratotic papules also were observed on the cheeks, axillae, chest, abdomen, back, lateral upper arms, and thighs. Dental examination revealed a large space between the upper anterior teeth and the lower anterior teeth. The upper anterior teeth were anteverted, there was congenital absence of right lower central incisors, and the anterior teeth were in deep overbite and overjet (Figure 1B). There was gingival atrophy and calculus dentalis in the upper and lower teeth. He had a fissured tongue with atrophic filiform papillae (Figure 1C).
Laboratory testing of the blood, urine, stool, hepatic and renal function, and serum vitamin B2 and B12 levelswere all within reference range. A panoramic radiograph of the occlusal surface showed congenital absence of right lower central incisors (Figure 2), and a lateral projection of a cranial radiograph confirmed that the anterior teeth were in deep overbite and overjet. Direct microscopy and fungal culture of material collected from the dorsal tongue were negative. Direct microscopy and fungal culture of diseased hairs also were negative. A rapid plasma reagin test, Treponema pallidum hemagglutination assay, and human immunodeficiency virus test were negative. Staphylococcus aureus was isolated from the scalp pustules, and in vitro drug susceptibility testing showed that it was sensitive to clarithromycin and moxifloxacin. Pathological examination of a biopsy of the occipital skin lesions showed a thickened epidermal spinous layer and massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles (Figure 3). Pathological examination of the skin lesions on the superciliary arch also showed infiltration of inflammatory cells in the dermis around the hair follicles.
Based on these findings, a diagnosis of folliculitis spinulosa decalvans (FSD) was made and the patient was started on clarithromycin (0.25 g twice daily), metronidazole (0.2 g 3 times daily), viaminate (50 mg 3 times daily), and fusidic acid cream (coating the affected area twice daily). When he returned for follow-up 1 month later, the pustules had disappeared and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.
Comment
Folliculitis spinulosa decalvans, along with keratosis follicularis spinulosa decalvans (KFSD), keratosis pilaris atrophicans faciei, and atrophoderma vermiculatum, belongs to a group of diseases that includes keratosis pilaris atrophicans. In 1994, Oranje et al1 suggested the term folliculitis spinulosa decalvans, with signs including persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp, which may be exacerbated at puberty. Staphylococcus aureus was isolated from the pustules in one study2; however, in another study, repeated cultures were negative.3 Although the main inheritance pattern of KFSD is X-linked, autosomal-dominant inheritance is more common in FSD. Furthermore, there are certain differences in the clinical manifestations of these 2 conditions. Therefore, it remains controversial if FSD is an independent disease or merely a subtype of KFSD.
Our patient’s symptoms manifested after puberty, primarily pustules as well as atrophic and scarring alopecia of the scalp and follicular keratotic papules on the head, face, trunk, lateral upper arms, and thighs. Pathologic examination showed massive infiltration of plasma cells, neutrophils, and multinucleated giant cells around the hair follicles. The clinical and histopathologic findings met the diagnostic criteria for FSD.
Folliculitis spinulosa decalvans is a rare clinical condition with few cases reported.3-5 In addition to the aforementioned characteristic clinical manifestations, our patient also had dental anomalies, a fissured tongue, and atrophy of the tongue papillae, which are not known to be associated with FSD. Dental anomalies are characteristic of patients with Down syndrome, ectodermal dysplasia, Papillon-Lefèvre syndrome, and other conditions.6 Fissured tongue is a normal variant that occurs in 5% to 11% of individuals. It also is a classic but nonspecific feature of Melkersson-Rosenthal syndrome and may occur in psoriasis, Down syndrome, acromegaly, and Sjögren syndrome.7 Atrophy of the tongue papillae is associated with anemia, pellagra, Sjögren syndrome, candidiasis, and other conditions.8 Because there are no known reports of associations between FSD and any of these oral manifestations, it is possible that they were unrelated to FSD in our patient.
Folliculitis spinulosa decalvans usually is recurrent and there is no consistently effective treatment for it. Kunte et al4 reported that dapsone (100 mg/d) led to resolution of scalp inflammation and pustules within 1 month. Romine et al2 reported that a 3-week course of dichloroxacillin (250 mg 4 times daily) induced disappearance of pustules around the hair follicles. However, Hallai et al5 reported a patient who was resistant to isotretinoin treatment. In our case, after 1 month of treatment with clarithromycin, metronidazole, viaminate, and fusidic acid cream, the pustules had resolved and the black scabs had fallen off, leaving atrophic scars. The long-term efficacy of this regimen is still under observation.
Conclusion
We report a case of FSD with dental anomalies, a fissured tongue, and atrophy of tongue papillae, none of which have previously been reported in association with FSD. We, therefore, believe that our patient’s oral manifestations are unrelated to FSD.
- Oranje AP, van Osch LD, Oosterwijk JC. Keratosis pilaris atrophicans. one heterogeneous disease or a symptom in different clinical entities? Arch Dermatol. 1994;13:500-502.
- Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris. keratosis follicularis spinulosa decalvans. Arch Dermatol. 1997;13:381-384.
- Di Lernia V, Ricci C. Folliculitis spinulosa decalvans: an uncommon entity within the keratosis pilaris atrophicans spectrum. Pediatr Dermatol. 2006;23:255-258.
- Kunte C, Loeser C, Wolff H. Folliculitis spinulosa decalvans: successful therapy with dapsone. J Am Acad Dermatol. 1998;39(5, pt 2):891-892.
- Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
- Scully C, Hegarty A. The oral cavity and lips. In: Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. 8th ed. Oxford, England: Wiley-Blackwell; 2010:69.7-69.10.
- Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Companies; 2007:643.
- Mulliken RA, Casner MJ. Oral manifestations of systemic disease. Emerg Med Clin North Am. 2000;18:565-575.
- Oranje AP, van Osch LD, Oosterwijk JC. Keratosis pilaris atrophicans. one heterogeneous disease or a symptom in different clinical entities? Arch Dermatol. 1994;13:500-502.
- Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris. keratosis follicularis spinulosa decalvans. Arch Dermatol. 1997;13:381-384.
- Di Lernia V, Ricci C. Folliculitis spinulosa decalvans: an uncommon entity within the keratosis pilaris atrophicans spectrum. Pediatr Dermatol. 2006;23:255-258.
- Kunte C, Loeser C, Wolff H. Folliculitis spinulosa decalvans: successful therapy with dapsone. J Am Acad Dermatol. 1998;39(5, pt 2):891-892.
- Hallai N, Thompson I, Williams P, et al. Folliculitis spinulosa decalvans: failure to respond to oral isotretinoin. J Eur Acad Dermatol Venereol. 2006;20:223-224.
- Scully C, Hegarty A. The oral cavity and lips. In: Burns T, Breathnach S, Cox N, et al. Rook’s Textbook of Dermatology. 8th ed. Oxford, England: Wiley-Blackwell; 2010:69.7-69.10.
- Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine. 7th ed. New York, NY: McGraw-Hill Companies; 2007:643.
- Mulliken RA, Casner MJ. Oral manifestations of systemic disease. Emerg Med Clin North Am. 2000;18:565-575.
Practice Points
- Folliculitis spinulosa decalvans (FSD) presents with persistent pustules, characteristic keratotic papules, and scarring alopecia of the scalp.
- In the case described here, oral manifestations also were present but are not characteristic of FSD.
Subungual Onycholemmal Cyst of the Toenail Mimicking Subungual Melanoma
Case Report
A 23-year-old woman presented with a horizontal split along the midline of the right great toenail associated with some tenderness of 2 to 3 months’ duration. Approximately 5 years prior, she noticed a bluish-colored area under the nail that had been steadily increasing in size. She denied a history of trauma, drainage, or bleeding. There was no history of other nail abnormalities. Her medications and personal, family, and social history were noncontributory.
Physical examination of the right great toenail revealed a horizontal split of the nail plate with a bluish hue visible under the nail plate (Figure 1A). The remaining toenails and fingernails were normal. A punch biopsy of the nail bed was performed with a presumptive clinical diagnosis of subungual melanoma versus melanocytic nevus versus cyst (Figure 1B). Nail plate avulsion revealed a blackened nail bed dotted with areas of bluish color and a red friable nodule present focally. Upon further inspection, extension was apparent into the distal matrix.
Histopathologic examination revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus catagen cyst admixed with the presence of both an intermittent focal granular layer and an eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC)(Figure 2). A reexcision was performed with removal of half of the nail bed, including a portion of the distal matrix extending inferiorly to the bone. Variably sized, epithelium-lined, keratin-filled cystic structures emanated from the nail bed epithelium. There were foci of hemorrhage and granulation tissue secondary to cyst rupture (Figure 3). The defect healed by secondary intention. No clinical evidence of recurrence was seen at 6-month follow-up.
Subungual onycholemmal cysts, also known as subungual epidermoid cysts or subungual epidermoid inclusions, are rare and distinctive nail abnormalities occurring in the dermis of the nail bed. We present a case of an SOC in a toenail mimicking subungual malignant melanoma.
Originally described by Samman1 in 1959, SOCs were attributed to trauma to the nail with resultant implantation of the epidermis into the deeper tissue. Lewin2,3 examined 90 postmortem fingernail and nail bed samples and found 8 subungual epidermoid cysts associated with clubbing of the fingernails. He postulated that the early pathogenesis of clubbing involved dermal fibroblast proliferation in the nail bed, leading to sequestration of nail bed epithelium into the dermis with resultant cyst formation. Microscopic subungual cysts also were identified in normal-appearing nails without evidence of trauma, thought to have arisen from the tips of the nail bed rete ridges by a process of bulbous proliferation rather than sequestration. These findings in normal nails suggest that SOCs may represent a more common entity than previously recognized.
It is imperative to recognize the presence of nail unit tumors early because of the risk for permanent nail plate dystrophy and the possibility of a malignant tumor.4,5 Subungual onycholemmal cysts may present with a wide spectrum of clinical findings including marked subungual hyperkeratosis, onychodystrophy, ridging, nail bed pigmentation, clubbing, thickening, or less often a normal-appearing nail. Based on reported cases, several trends are evident. Although nail dystrophy is most often asymptomatic, pain is not uncommon.5,6 It most commonly involves single digits, predominantly thumbs and great toenails.7,8 This predilection suggests that trauma or other local factors may be involved in its pathogenesis. Of note, trauma to the nail may occur years before the development of the lesions or it may not be recalled at all.
Diagnosis requires a degree of clinical suspicion and a nail bed biopsy with partial or total nail plate avulsion to visualize the pathologic portion of the nail bed. Because surgical intervention may lead to the implantation of epithelium, recurrences after nail biopsy or excision may occur.
In contrast to epidermal inclusion cysts arising in the skin, most SOCs do not have a granular layer.9 Hair and nails represent analogous differentiation products of the ectoderm. The nail matrix is homologous to portions of the hair matrix, while the nail bed epithelium is comparable to the outer root sheath of the hair follicle.7 Subungual onycholemmal cysts originate from the nail bed epithelium, which keratinizes in the absence of a granular layer, similar to the follicular isthmus outer root sheath. Thus, SOCs are comparable to the outer root sheath–derived isthmus-catagen cysts because of their abrupt central keratinization.8
Subungual onycholemmal cysts also must be distinguished from slowly growing malignant tumors of the nail bed epithelium, referred to as onycholemmal carcinomas by Alessi et al.10 This entity characteristically presents in elderly patients as a slowly growing, circumscribed, subungual discoloration that may ulcerate, destroying the nail apparatus and penetrating the phalangeal bone. On histopathology, it is characterized by small cysts filled with eosinophilic keratin devoid of a granular layer and lined by atypical squamous epithelium accompanied by solid nests and strands of atypical keratinocytes within the dermis.11 When a cystic component and clear cells predominate, the designation of malignant proliferating onycholemmal cyst has been applied. Its infiltrative growth pattern with destruction of the underlying bone makes it an important entity to exclude when considering the differential diagnosis of tumors of the nail bed.
Subungual melanomas comprise only 1% to 3% of malignant melanomas and 85% are initially misdiagnosed due to their rarity and nonspecific variable presentation. Aside from clinical evidence of Hutchinson sign in the early stages in almost all cases, accurate diagnosis of subungual melanoma and differentiation from SOCs relies on histopathology. A biopsy is necessary to make the diagnosis, but even microscopic findings may be nonspecific during the early stages.
Conclusion
We report a case of a 23-year-old woman with horizontal ridging and tenderness of the right great toenail associated with pigmentation of 5 years’ duration due to an SOC. The etiology of these subungual cysts, with or without nail abnormalities, still remains unclear. Its predilection for the thumbs and great toenails suggests that trauma or other local factors may be involved in its pathogenesis. Because of the rarity of this entity, there are no guidelines for surgical treatment. Subungual onycholemmal cysts may be an underrecognized and more common entity that must be considered when discussing tumors of the nail unit.
- Samman PD. The human toe nail. its genesis and blood supply. Br J Dermatol. 1959;71:296-302.
- Lewin K. The normal fingernail. Br J Dermatol. 1965;77:421-430.
- Lewin K. Subungual epidermoid inclusions. Br J Dermatol. 1969;81:671-675.
- Dominguez-Cherit J, Chanussot-Deprez C, Maria-Sarti H, et al. Nail unit tumors: a study of 234 patients in the dermatology department of the “Dr. Manuel Gea González” General Hospital in Mexico City. Dermatol Surg. 2008;34:1363-1371.
- Sáez-de-Ocariz MM, Domínguez-Cherit J, García-Corona C. Subungual epidermoid cysts. Int J Dermatol. 2001;40:524-526.
- Molly DO, Herbert K. Subungual epidermoid cyst. J Hand Surg Br. 2006;31:345.
- Telang GH, Jellinek N. Multiple calcified subungual epidermoid inclusions. J Am Acad Dermatol. 2007;56:336-339.
- Fanti PA, Tosti A. Subungual epidermoid inclusions: report of 8 cases. Dermatologica. 1989;178:209-212.
- Takiyoshi N, Nakano H, Matsuzaki T, et al. An eclipse in the subungual space: a diagnostic sign for a subungual epidermal cyst? Br J Dermatol. 2009;161:962-963.
- Alessi E, Coggi A, Gianotti R, et al. Onycholemmal carcinoma. Am J Dermatopathol. 2004;26:397-402.
- Inaoki M, Makino E, Adachi M, et al. Onycholemmal carcinoma. J Cutan Pathol. 2006;33:577-580.
Case Report
A 23-year-old woman presented with a horizontal split along the midline of the right great toenail associated with some tenderness of 2 to 3 months’ duration. Approximately 5 years prior, she noticed a bluish-colored area under the nail that had been steadily increasing in size. She denied a history of trauma, drainage, or bleeding. There was no history of other nail abnormalities. Her medications and personal, family, and social history were noncontributory.
Physical examination of the right great toenail revealed a horizontal split of the nail plate with a bluish hue visible under the nail plate (Figure 1A). The remaining toenails and fingernails were normal. A punch biopsy of the nail bed was performed with a presumptive clinical diagnosis of subungual melanoma versus melanocytic nevus versus cyst (Figure 1B). Nail plate avulsion revealed a blackened nail bed dotted with areas of bluish color and a red friable nodule present focally. Upon further inspection, extension was apparent into the distal matrix.
Histopathologic examination revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus catagen cyst admixed with the presence of both an intermittent focal granular layer and an eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC)(Figure 2). A reexcision was performed with removal of half of the nail bed, including a portion of the distal matrix extending inferiorly to the bone. Variably sized, epithelium-lined, keratin-filled cystic structures emanated from the nail bed epithelium. There were foci of hemorrhage and granulation tissue secondary to cyst rupture (Figure 3). The defect healed by secondary intention. No clinical evidence of recurrence was seen at 6-month follow-up.
Subungual onycholemmal cysts, also known as subungual epidermoid cysts or subungual epidermoid inclusions, are rare and distinctive nail abnormalities occurring in the dermis of the nail bed. We present a case of an SOC in a toenail mimicking subungual malignant melanoma.
Originally described by Samman1 in 1959, SOCs were attributed to trauma to the nail with resultant implantation of the epidermis into the deeper tissue. Lewin2,3 examined 90 postmortem fingernail and nail bed samples and found 8 subungual epidermoid cysts associated with clubbing of the fingernails. He postulated that the early pathogenesis of clubbing involved dermal fibroblast proliferation in the nail bed, leading to sequestration of nail bed epithelium into the dermis with resultant cyst formation. Microscopic subungual cysts also were identified in normal-appearing nails without evidence of trauma, thought to have arisen from the tips of the nail bed rete ridges by a process of bulbous proliferation rather than sequestration. These findings in normal nails suggest that SOCs may represent a more common entity than previously recognized.
It is imperative to recognize the presence of nail unit tumors early because of the risk for permanent nail plate dystrophy and the possibility of a malignant tumor.4,5 Subungual onycholemmal cysts may present with a wide spectrum of clinical findings including marked subungual hyperkeratosis, onychodystrophy, ridging, nail bed pigmentation, clubbing, thickening, or less often a normal-appearing nail. Based on reported cases, several trends are evident. Although nail dystrophy is most often asymptomatic, pain is not uncommon.5,6 It most commonly involves single digits, predominantly thumbs and great toenails.7,8 This predilection suggests that trauma or other local factors may be involved in its pathogenesis. Of note, trauma to the nail may occur years before the development of the lesions or it may not be recalled at all.
Diagnosis requires a degree of clinical suspicion and a nail bed biopsy with partial or total nail plate avulsion to visualize the pathologic portion of the nail bed. Because surgical intervention may lead to the implantation of epithelium, recurrences after nail biopsy or excision may occur.
In contrast to epidermal inclusion cysts arising in the skin, most SOCs do not have a granular layer.9 Hair and nails represent analogous differentiation products of the ectoderm. The nail matrix is homologous to portions of the hair matrix, while the nail bed epithelium is comparable to the outer root sheath of the hair follicle.7 Subungual onycholemmal cysts originate from the nail bed epithelium, which keratinizes in the absence of a granular layer, similar to the follicular isthmus outer root sheath. Thus, SOCs are comparable to the outer root sheath–derived isthmus-catagen cysts because of their abrupt central keratinization.8
Subungual onycholemmal cysts also must be distinguished from slowly growing malignant tumors of the nail bed epithelium, referred to as onycholemmal carcinomas by Alessi et al.10 This entity characteristically presents in elderly patients as a slowly growing, circumscribed, subungual discoloration that may ulcerate, destroying the nail apparatus and penetrating the phalangeal bone. On histopathology, it is characterized by small cysts filled with eosinophilic keratin devoid of a granular layer and lined by atypical squamous epithelium accompanied by solid nests and strands of atypical keratinocytes within the dermis.11 When a cystic component and clear cells predominate, the designation of malignant proliferating onycholemmal cyst has been applied. Its infiltrative growth pattern with destruction of the underlying bone makes it an important entity to exclude when considering the differential diagnosis of tumors of the nail bed.
Subungual melanomas comprise only 1% to 3% of malignant melanomas and 85% are initially misdiagnosed due to their rarity and nonspecific variable presentation. Aside from clinical evidence of Hutchinson sign in the early stages in almost all cases, accurate diagnosis of subungual melanoma and differentiation from SOCs relies on histopathology. A biopsy is necessary to make the diagnosis, but even microscopic findings may be nonspecific during the early stages.
Conclusion
We report a case of a 23-year-old woman with horizontal ridging and tenderness of the right great toenail associated with pigmentation of 5 years’ duration due to an SOC. The etiology of these subungual cysts, with or without nail abnormalities, still remains unclear. Its predilection for the thumbs and great toenails suggests that trauma or other local factors may be involved in its pathogenesis. Because of the rarity of this entity, there are no guidelines for surgical treatment. Subungual onycholemmal cysts may be an underrecognized and more common entity that must be considered when discussing tumors of the nail unit.
Case Report
A 23-year-old woman presented with a horizontal split along the midline of the right great toenail associated with some tenderness of 2 to 3 months’ duration. Approximately 5 years prior, she noticed a bluish-colored area under the nail that had been steadily increasing in size. She denied a history of trauma, drainage, or bleeding. There was no history of other nail abnormalities. Her medications and personal, family, and social history were noncontributory.
Physical examination of the right great toenail revealed a horizontal split of the nail plate with a bluish hue visible under the nail plate (Figure 1A). The remaining toenails and fingernails were normal. A punch biopsy of the nail bed was performed with a presumptive clinical diagnosis of subungual melanoma versus melanocytic nevus versus cyst (Figure 1B). Nail plate avulsion revealed a blackened nail bed dotted with areas of bluish color and a red friable nodule present focally. Upon further inspection, extension was apparent into the distal matrix.
Histopathologic examination revealed a cystic structure with an epithelial lining mostly reminiscent of an isthmus catagen cyst admixed with the presence of both an intermittent focal granular layer and an eosinophilic cuticle surrounding pink laminated keratin, most consistent with a diagnosis of subungual onycholemmal cyst (SOC)(Figure 2). A reexcision was performed with removal of half of the nail bed, including a portion of the distal matrix extending inferiorly to the bone. Variably sized, epithelium-lined, keratin-filled cystic structures emanated from the nail bed epithelium. There were foci of hemorrhage and granulation tissue secondary to cyst rupture (Figure 3). The defect healed by secondary intention. No clinical evidence of recurrence was seen at 6-month follow-up.
Subungual onycholemmal cysts, also known as subungual epidermoid cysts or subungual epidermoid inclusions, are rare and distinctive nail abnormalities occurring in the dermis of the nail bed. We present a case of an SOC in a toenail mimicking subungual malignant melanoma.
Originally described by Samman1 in 1959, SOCs were attributed to trauma to the nail with resultant implantation of the epidermis into the deeper tissue. Lewin2,3 examined 90 postmortem fingernail and nail bed samples and found 8 subungual epidermoid cysts associated with clubbing of the fingernails. He postulated that the early pathogenesis of clubbing involved dermal fibroblast proliferation in the nail bed, leading to sequestration of nail bed epithelium into the dermis with resultant cyst formation. Microscopic subungual cysts also were identified in normal-appearing nails without evidence of trauma, thought to have arisen from the tips of the nail bed rete ridges by a process of bulbous proliferation rather than sequestration. These findings in normal nails suggest that SOCs may represent a more common entity than previously recognized.
It is imperative to recognize the presence of nail unit tumors early because of the risk for permanent nail plate dystrophy and the possibility of a malignant tumor.4,5 Subungual onycholemmal cysts may present with a wide spectrum of clinical findings including marked subungual hyperkeratosis, onychodystrophy, ridging, nail bed pigmentation, clubbing, thickening, or less often a normal-appearing nail. Based on reported cases, several trends are evident. Although nail dystrophy is most often asymptomatic, pain is not uncommon.5,6 It most commonly involves single digits, predominantly thumbs and great toenails.7,8 This predilection suggests that trauma or other local factors may be involved in its pathogenesis. Of note, trauma to the nail may occur years before the development of the lesions or it may not be recalled at all.
Diagnosis requires a degree of clinical suspicion and a nail bed biopsy with partial or total nail plate avulsion to visualize the pathologic portion of the nail bed. Because surgical intervention may lead to the implantation of epithelium, recurrences after nail biopsy or excision may occur.
In contrast to epidermal inclusion cysts arising in the skin, most SOCs do not have a granular layer.9 Hair and nails represent analogous differentiation products of the ectoderm. The nail matrix is homologous to portions of the hair matrix, while the nail bed epithelium is comparable to the outer root sheath of the hair follicle.7 Subungual onycholemmal cysts originate from the nail bed epithelium, which keratinizes in the absence of a granular layer, similar to the follicular isthmus outer root sheath. Thus, SOCs are comparable to the outer root sheath–derived isthmus-catagen cysts because of their abrupt central keratinization.8
Subungual onycholemmal cysts also must be distinguished from slowly growing malignant tumors of the nail bed epithelium, referred to as onycholemmal carcinomas by Alessi et al.10 This entity characteristically presents in elderly patients as a slowly growing, circumscribed, subungual discoloration that may ulcerate, destroying the nail apparatus and penetrating the phalangeal bone. On histopathology, it is characterized by small cysts filled with eosinophilic keratin devoid of a granular layer and lined by atypical squamous epithelium accompanied by solid nests and strands of atypical keratinocytes within the dermis.11 When a cystic component and clear cells predominate, the designation of malignant proliferating onycholemmal cyst has been applied. Its infiltrative growth pattern with destruction of the underlying bone makes it an important entity to exclude when considering the differential diagnosis of tumors of the nail bed.
Subungual melanomas comprise only 1% to 3% of malignant melanomas and 85% are initially misdiagnosed due to their rarity and nonspecific variable presentation. Aside from clinical evidence of Hutchinson sign in the early stages in almost all cases, accurate diagnosis of subungual melanoma and differentiation from SOCs relies on histopathology. A biopsy is necessary to make the diagnosis, but even microscopic findings may be nonspecific during the early stages.
Conclusion
We report a case of a 23-year-old woman with horizontal ridging and tenderness of the right great toenail associated with pigmentation of 5 years’ duration due to an SOC. The etiology of these subungual cysts, with or without nail abnormalities, still remains unclear. Its predilection for the thumbs and great toenails suggests that trauma or other local factors may be involved in its pathogenesis. Because of the rarity of this entity, there are no guidelines for surgical treatment. Subungual onycholemmal cysts may be an underrecognized and more common entity that must be considered when discussing tumors of the nail unit.
- Samman PD. The human toe nail. its genesis and blood supply. Br J Dermatol. 1959;71:296-302.
- Lewin K. The normal fingernail. Br J Dermatol. 1965;77:421-430.
- Lewin K. Subungual epidermoid inclusions. Br J Dermatol. 1969;81:671-675.
- Dominguez-Cherit J, Chanussot-Deprez C, Maria-Sarti H, et al. Nail unit tumors: a study of 234 patients in the dermatology department of the “Dr. Manuel Gea González” General Hospital in Mexico City. Dermatol Surg. 2008;34:1363-1371.
- Sáez-de-Ocariz MM, Domínguez-Cherit J, García-Corona C. Subungual epidermoid cysts. Int J Dermatol. 2001;40:524-526.
- Molly DO, Herbert K. Subungual epidermoid cyst. J Hand Surg Br. 2006;31:345.
- Telang GH, Jellinek N. Multiple calcified subungual epidermoid inclusions. J Am Acad Dermatol. 2007;56:336-339.
- Fanti PA, Tosti A. Subungual epidermoid inclusions: report of 8 cases. Dermatologica. 1989;178:209-212.
- Takiyoshi N, Nakano H, Matsuzaki T, et al. An eclipse in the subungual space: a diagnostic sign for a subungual epidermal cyst? Br J Dermatol. 2009;161:962-963.
- Alessi E, Coggi A, Gianotti R, et al. Onycholemmal carcinoma. Am J Dermatopathol. 2004;26:397-402.
- Inaoki M, Makino E, Adachi M, et al. Onycholemmal carcinoma. J Cutan Pathol. 2006;33:577-580.
- Samman PD. The human toe nail. its genesis and blood supply. Br J Dermatol. 1959;71:296-302.
- Lewin K. The normal fingernail. Br J Dermatol. 1965;77:421-430.
- Lewin K. Subungual epidermoid inclusions. Br J Dermatol. 1969;81:671-675.
- Dominguez-Cherit J, Chanussot-Deprez C, Maria-Sarti H, et al. Nail unit tumors: a study of 234 patients in the dermatology department of the “Dr. Manuel Gea González” General Hospital in Mexico City. Dermatol Surg. 2008;34:1363-1371.
- Sáez-de-Ocariz MM, Domínguez-Cherit J, García-Corona C. Subungual epidermoid cysts. Int J Dermatol. 2001;40:524-526.
- Molly DO, Herbert K. Subungual epidermoid cyst. J Hand Surg Br. 2006;31:345.
- Telang GH, Jellinek N. Multiple calcified subungual epidermoid inclusions. J Am Acad Dermatol. 2007;56:336-339.
- Fanti PA, Tosti A. Subungual epidermoid inclusions: report of 8 cases. Dermatologica. 1989;178:209-212.
- Takiyoshi N, Nakano H, Matsuzaki T, et al. An eclipse in the subungual space: a diagnostic sign for a subungual epidermal cyst? Br J Dermatol. 2009;161:962-963.
- Alessi E, Coggi A, Gianotti R, et al. Onycholemmal carcinoma. Am J Dermatopathol. 2004;26:397-402.
- Inaoki M, Makino E, Adachi M, et al. Onycholemmal carcinoma. J Cutan Pathol. 2006;33:577-580.
Practice Points
- Trauma to the nail may occur years before the development of subungual onycholemmal cysts or it may not be recalled at all.
- Diagnosis requires a degree of clinical suspicion and a nail bed biopsy.
- Subungual onycholemmal cysts must be distinguished from slowly growing malignant tumors of the nail bed epithelium.
Tofacitinib clears pediatric alopecia areata in small study
MINNEAPOLIS – The first study to evaluate tofacitinib’s effectiveness at treating severe alopecia areata in the pediatric population found that the janus kinase inhibitor was effective for more than half of the patients, and well tolerated by all.
Of a case series of 13 pediatric patients who had alopecia areata (AA) and were treated with tofacitinib, 9 (68%) experienced “clinically significant” regrowth of hair, with mean improvement in the Severity of Alopecia Tool (SALT) score of 88% for these responders. The nonresponding group, all of whom had alopecia universalis or totalis, saw essentially no response, with a 1% reduction in SALT score.
Lucy Y. Liu, a medical student at Yale University, New Haven, Conn., presented the findings at the annual meeting of the Society for Pediatric Dermatology.
Ms. Liu and her coinvestigators reported that all of the patients had severe AA by SALT scoring, with an overall mean pretreatment SALT score of 74. Eight of the patients (62%) had alopecia universalis, and two (15%) had alopecia totalis.
The patients ranged in age from 12 to 17 years, with a median age of 15. All but three were male, and patients were an average 9 years old at onset of AA. For patients with alopecia totalis or universalis, the duration of the current episode was a median 1.75 years.
Five patients (38%) had atopic dermatitis, while 1 (8%) had thyroid disease. Three patients (23%) had family members with AA; all but one patient, however, had a family history of autoimmune disease of some sort.
Patients were given tofacitinib 5 mg orally twice daily for 5 months. One patient developed new patches of alopecia during treatment, so the dosing for that patient was increased to 10 mg/5 mg daily.
Adverse events for participants included headaches, upper respiratory infections, and “mild, transient increases in transaminases,” wrote Dr. Lieu and her collaborators. No serious adverse events were reported.
Previous work at Yale had shown that tofacitinib reversed alopecia universalis in a patient who received the medication for plaque psoriasis, and that topical treatment with ruxolitinib, another janus kinase inhibitor, was effective in treating alopecia universalis.
Study limitations included the small sample size and the relatively short duration of follow-up, an important consideration because relapse has been observed after tofacitinib treatment in AA. Still, “Tofacitinib is a promising therapy for the treatment of severe AA in adolescents,” wrote Ms. Liu and her colleagues, recommending randomized clinical trials for further exploration of efficacy and safety in the pediatric population.
On Twitter @karioakes
MINNEAPOLIS – The first study to evaluate tofacitinib’s effectiveness at treating severe alopecia areata in the pediatric population found that the janus kinase inhibitor was effective for more than half of the patients, and well tolerated by all.
Of a case series of 13 pediatric patients who had alopecia areata (AA) and were treated with tofacitinib, 9 (68%) experienced “clinically significant” regrowth of hair, with mean improvement in the Severity of Alopecia Tool (SALT) score of 88% for these responders. The nonresponding group, all of whom had alopecia universalis or totalis, saw essentially no response, with a 1% reduction in SALT score.
Lucy Y. Liu, a medical student at Yale University, New Haven, Conn., presented the findings at the annual meeting of the Society for Pediatric Dermatology.
Ms. Liu and her coinvestigators reported that all of the patients had severe AA by SALT scoring, with an overall mean pretreatment SALT score of 74. Eight of the patients (62%) had alopecia universalis, and two (15%) had alopecia totalis.
The patients ranged in age from 12 to 17 years, with a median age of 15. All but three were male, and patients were an average 9 years old at onset of AA. For patients with alopecia totalis or universalis, the duration of the current episode was a median 1.75 years.
Five patients (38%) had atopic dermatitis, while 1 (8%) had thyroid disease. Three patients (23%) had family members with AA; all but one patient, however, had a family history of autoimmune disease of some sort.
Patients were given tofacitinib 5 mg orally twice daily for 5 months. One patient developed new patches of alopecia during treatment, so the dosing for that patient was increased to 10 mg/5 mg daily.
Adverse events for participants included headaches, upper respiratory infections, and “mild, transient increases in transaminases,” wrote Dr. Lieu and her collaborators. No serious adverse events were reported.
Previous work at Yale had shown that tofacitinib reversed alopecia universalis in a patient who received the medication for plaque psoriasis, and that topical treatment with ruxolitinib, another janus kinase inhibitor, was effective in treating alopecia universalis.
Study limitations included the small sample size and the relatively short duration of follow-up, an important consideration because relapse has been observed after tofacitinib treatment in AA. Still, “Tofacitinib is a promising therapy for the treatment of severe AA in adolescents,” wrote Ms. Liu and her colleagues, recommending randomized clinical trials for further exploration of efficacy and safety in the pediatric population.
On Twitter @karioakes
MINNEAPOLIS – The first study to evaluate tofacitinib’s effectiveness at treating severe alopecia areata in the pediatric population found that the janus kinase inhibitor was effective for more than half of the patients, and well tolerated by all.
Of a case series of 13 pediatric patients who had alopecia areata (AA) and were treated with tofacitinib, 9 (68%) experienced “clinically significant” regrowth of hair, with mean improvement in the Severity of Alopecia Tool (SALT) score of 88% for these responders. The nonresponding group, all of whom had alopecia universalis or totalis, saw essentially no response, with a 1% reduction in SALT score.
Lucy Y. Liu, a medical student at Yale University, New Haven, Conn., presented the findings at the annual meeting of the Society for Pediatric Dermatology.
Ms. Liu and her coinvestigators reported that all of the patients had severe AA by SALT scoring, with an overall mean pretreatment SALT score of 74. Eight of the patients (62%) had alopecia universalis, and two (15%) had alopecia totalis.
The patients ranged in age from 12 to 17 years, with a median age of 15. All but three were male, and patients were an average 9 years old at onset of AA. For patients with alopecia totalis or universalis, the duration of the current episode was a median 1.75 years.
Five patients (38%) had atopic dermatitis, while 1 (8%) had thyroid disease. Three patients (23%) had family members with AA; all but one patient, however, had a family history of autoimmune disease of some sort.
Patients were given tofacitinib 5 mg orally twice daily for 5 months. One patient developed new patches of alopecia during treatment, so the dosing for that patient was increased to 10 mg/5 mg daily.
Adverse events for participants included headaches, upper respiratory infections, and “mild, transient increases in transaminases,” wrote Dr. Lieu and her collaborators. No serious adverse events were reported.
Previous work at Yale had shown that tofacitinib reversed alopecia universalis in a patient who received the medication for plaque psoriasis, and that topical treatment with ruxolitinib, another janus kinase inhibitor, was effective in treating alopecia universalis.
Study limitations included the small sample size and the relatively short duration of follow-up, an important consideration because relapse has been observed after tofacitinib treatment in AA. Still, “Tofacitinib is a promising therapy for the treatment of severe AA in adolescents,” wrote Ms. Liu and her colleagues, recommending randomized clinical trials for further exploration of efficacy and safety in the pediatric population.
On Twitter @karioakes
AT THE SPD ANNUAL MEETING
Key clinical point: The JAK inhibitor tofacitinib resolved alopecia areata in 68% of pediatric patients.
Major finding: Among the responders, the Severity of Alopecia Tool score improved by a mean 88% over 5 months.
Data source: Case series of 13 pediatric patients with severe alopecia areata treated with tofacitinib at a single site.
Disclosures: Ms. Liu and her collaborators reported no conflicts of interest.
What’s Eating You? Tick Bite Alopecia
Case Report
A 44-year-old woman presented with a localized patch of hair loss on the frontal scalp of several month’s duration. She had been bitten by a tick at this site during the summer. Two months later
A punch biopsy was obtained from an indurated area of hyperpigmentation adjacent to the eschar. Both vertical and horizontal sections were obtained, revealing a relatively normal epidermis, a marked decrease in follicular structures with loss of sebaceous glands, and dense perifollicular lymphocytic inflammation with a few scattered eosinophils (Figures 2 and 3).
Historical Perspective
Tick bite alopecia was first described in the French literature in 19211 and in the English-language literature in 1955.2 A few additional cases were subsequently reported.3-5 In 2008, Castelli et al6 described the histologic and immunohistochemical features of 25 tick bite cases, a few of which resulted in alopecia. Other than these reports, little original information has been written about tick bite alopecia.
Clinical and Histologic Presentation
Tick bite alopecia is well described in the veterinary literature.7-9 It is possible that the condition is underreported in humans because the cause is often obvious or the alopecia is never discovered. The typical presentation is a roughly oval zone of alopecia that develops 1 to 2 weeks after the removal of a tick from the scalp. Often there is a small central eschar representing the site of tick attachment and the surrounding scalp may appear scaly. In one report of 2 siblings, multiple oval zones of alopecia resembling the moth-eaten alopecia of syphilis were noted in both patients, but only a single attached tick was found.2 In some reported cases, hair loss was only temporary, and at least partial if not complete regrowth of hair occurred.3,4 Follow-up on most cases is not provided, but to our knowledge permanent alopecia has not been described.
Information about the histologic findings of tick bite alopecia is particularly limited. In a report by Heyl,3 biopsies were conducted in 2 patients, but the areas selected for biopsy were the sites of tick attachment. Centrally dense, acute, and chronic inflammation was seen, as well as marked tissue necrosis of the connective tissue and hair follicles. Peripheral to the attachment zone, tissue necrosis was not found, but telogen hairs with “crumpled up hair shafts” were present.3 The histologic findings presented by Castelli et al6 were based on a single case of tick bite alopecia; however, the specimen was a generous excisional biopsy, allowing for a panoramic histologic view of the lesion. In the center of the specimen, hair follicles were absent, but residual follicular streamers and follicular remnants were surrounded by lymphocytic inflammation. Sebaceous glands were conspicuously absent, but foci with naked hairs, fibrosis, and granulomatous inflammation were seen. Peripherally, the hair follicles were thinned and miniaturized with an increased number of catagen/telogen hairs. Some follicles showed lamellar fibroplasia and perifollicular chronic inflammation. The inflammatory infiltrate consisted predominantly of helper T cells with a smaller population of B lymphocytes and a few plasma cells.6 In 2016, Lynch et al5 described a single case of tick bite alopecia and noted pseudolymphomatous inflammation with germinal center formation associated with hair miniaturization and an elevated catagen/telogen count; focal follicular mucinosis also was noted.Our histologic findings are similar to those of Castelli et al,6 except that the inflammatory infiltrate was clearly B-cell dominant, with a suggestion of germinal center formation, as noted by Lynch et al.5 This inflammatory pattern often can be encountered in a chronic tick bite lesion. Destruction of follicles and associated sebaceous glands and their replacement by follicular scars indicate that at least in the central portion of the lesion some permanent hair loss occurs. The presence of catagen/telogen hairs and miniaturized follicles indicates the potential for at least partial regrowth.
Similar to other investigators who have described tick bite alopecia, we can only speculate as to the mechanism by which clinical alopecia occurs. Given the density of the inflammatory infiltrate and perifollicular inflammation, it seems reasonable to assume that inflammation either destroys hair follicles or precipitates the catagen/telogen phase, resulting in temporary hair loss. The inflammation itself may be due to the presence of tick parts or the antigens in their saliva (or both). The delay between tick attachment and the onset of alopecia can be attributed to the time it takes follicles to cycle into the catagen/telogen phase and shed the hair shaft.
- Sauphar L. Alopecie peladoide consecutive a une piqure de tique. Bull Soc Fr Dermatol Syphiligr. 1921;28:442.
- Ross MS, Friede H. Alopecia due to tick bite. AMA Arch Derm. 1955;71:524-525.
- Heyl T. Tick bite alopecia. Clin Exp Dermatol. 1982;7:537-542.
- Marshall J. Alopecia after tick bite. S Afr Med J. 1966;40:555-556.
- Lynch MC, Milchak MA, Parnes H, et al. Tick bite alopecia: a report and review [published online April 19, 2016]. Am J Dermatopathol. doi:10.1097/DAD.0000000000000598.
- Castelli E, Caputo V, Morello V, et al. Local reactions to tick bites. Am J Dermatopathol. 2008;30:241-248.
- Nemeth NM, Ruder MG, Gerhold RW, et al. Demodectic mange, dermatophilosis, and other parasitic and bacterial dermatologic diseases in free-ranging white-tailed deer (Odocoileus virginianus) in the United States from 1975 to 2012. Vet Pathol. 2014;51:633-640.
- Welch DA, Samuel WM, Hudson RJ. Bioenergetic consequences of alopecia induced by Dermacentor albipictus (Acari: Ixodidae) on moose. J Med Entomol. 1990;27:656-660.
- Samuel WM. Locations of moose in northwestern Canada with hair loss probably caused by the winter tick, Dermacentor albipictus (Acari: Ixodidae). J Wildl Dis. 1989;25:436-439.
Case Report
A 44-year-old woman presented with a localized patch of hair loss on the frontal scalp of several month’s duration. She had been bitten by a tick at this site during the summer. Two months later
A punch biopsy was obtained from an indurated area of hyperpigmentation adjacent to the eschar. Both vertical and horizontal sections were obtained, revealing a relatively normal epidermis, a marked decrease in follicular structures with loss of sebaceous glands, and dense perifollicular lymphocytic inflammation with a few scattered eosinophils (Figures 2 and 3).
Historical Perspective
Tick bite alopecia was first described in the French literature in 19211 and in the English-language literature in 1955.2 A few additional cases were subsequently reported.3-5 In 2008, Castelli et al6 described the histologic and immunohistochemical features of 25 tick bite cases, a few of which resulted in alopecia. Other than these reports, little original information has been written about tick bite alopecia.
Clinical and Histologic Presentation
Tick bite alopecia is well described in the veterinary literature.7-9 It is possible that the condition is underreported in humans because the cause is often obvious or the alopecia is never discovered. The typical presentation is a roughly oval zone of alopecia that develops 1 to 2 weeks after the removal of a tick from the scalp. Often there is a small central eschar representing the site of tick attachment and the surrounding scalp may appear scaly. In one report of 2 siblings, multiple oval zones of alopecia resembling the moth-eaten alopecia of syphilis were noted in both patients, but only a single attached tick was found.2 In some reported cases, hair loss was only temporary, and at least partial if not complete regrowth of hair occurred.3,4 Follow-up on most cases is not provided, but to our knowledge permanent alopecia has not been described.
Information about the histologic findings of tick bite alopecia is particularly limited. In a report by Heyl,3 biopsies were conducted in 2 patients, but the areas selected for biopsy were the sites of tick attachment. Centrally dense, acute, and chronic inflammation was seen, as well as marked tissue necrosis of the connective tissue and hair follicles. Peripheral to the attachment zone, tissue necrosis was not found, but telogen hairs with “crumpled up hair shafts” were present.3 The histologic findings presented by Castelli et al6 were based on a single case of tick bite alopecia; however, the specimen was a generous excisional biopsy, allowing for a panoramic histologic view of the lesion. In the center of the specimen, hair follicles were absent, but residual follicular streamers and follicular remnants were surrounded by lymphocytic inflammation. Sebaceous glands were conspicuously absent, but foci with naked hairs, fibrosis, and granulomatous inflammation were seen. Peripherally, the hair follicles were thinned and miniaturized with an increased number of catagen/telogen hairs. Some follicles showed lamellar fibroplasia and perifollicular chronic inflammation. The inflammatory infiltrate consisted predominantly of helper T cells with a smaller population of B lymphocytes and a few plasma cells.6 In 2016, Lynch et al5 described a single case of tick bite alopecia and noted pseudolymphomatous inflammation with germinal center formation associated with hair miniaturization and an elevated catagen/telogen count; focal follicular mucinosis also was noted.Our histologic findings are similar to those of Castelli et al,6 except that the inflammatory infiltrate was clearly B-cell dominant, with a suggestion of germinal center formation, as noted by Lynch et al.5 This inflammatory pattern often can be encountered in a chronic tick bite lesion. Destruction of follicles and associated sebaceous glands and their replacement by follicular scars indicate that at least in the central portion of the lesion some permanent hair loss occurs. The presence of catagen/telogen hairs and miniaturized follicles indicates the potential for at least partial regrowth.
Similar to other investigators who have described tick bite alopecia, we can only speculate as to the mechanism by which clinical alopecia occurs. Given the density of the inflammatory infiltrate and perifollicular inflammation, it seems reasonable to assume that inflammation either destroys hair follicles or precipitates the catagen/telogen phase, resulting in temporary hair loss. The inflammation itself may be due to the presence of tick parts or the antigens in their saliva (or both). The delay between tick attachment and the onset of alopecia can be attributed to the time it takes follicles to cycle into the catagen/telogen phase and shed the hair shaft.
Case Report
A 44-year-old woman presented with a localized patch of hair loss on the frontal scalp of several month’s duration. She had been bitten by a tick at this site during the summer. Two months later
A punch biopsy was obtained from an indurated area of hyperpigmentation adjacent to the eschar. Both vertical and horizontal sections were obtained, revealing a relatively normal epidermis, a marked decrease in follicular structures with loss of sebaceous glands, and dense perifollicular lymphocytic inflammation with a few scattered eosinophils (Figures 2 and 3).
Historical Perspective
Tick bite alopecia was first described in the French literature in 19211 and in the English-language literature in 1955.2 A few additional cases were subsequently reported.3-5 In 2008, Castelli et al6 described the histologic and immunohistochemical features of 25 tick bite cases, a few of which resulted in alopecia. Other than these reports, little original information has been written about tick bite alopecia.
Clinical and Histologic Presentation
Tick bite alopecia is well described in the veterinary literature.7-9 It is possible that the condition is underreported in humans because the cause is often obvious or the alopecia is never discovered. The typical presentation is a roughly oval zone of alopecia that develops 1 to 2 weeks after the removal of a tick from the scalp. Often there is a small central eschar representing the site of tick attachment and the surrounding scalp may appear scaly. In one report of 2 siblings, multiple oval zones of alopecia resembling the moth-eaten alopecia of syphilis were noted in both patients, but only a single attached tick was found.2 In some reported cases, hair loss was only temporary, and at least partial if not complete regrowth of hair occurred.3,4 Follow-up on most cases is not provided, but to our knowledge permanent alopecia has not been described.
Information about the histologic findings of tick bite alopecia is particularly limited. In a report by Heyl,3 biopsies were conducted in 2 patients, but the areas selected for biopsy were the sites of tick attachment. Centrally dense, acute, and chronic inflammation was seen, as well as marked tissue necrosis of the connective tissue and hair follicles. Peripheral to the attachment zone, tissue necrosis was not found, but telogen hairs with “crumpled up hair shafts” were present.3 The histologic findings presented by Castelli et al6 were based on a single case of tick bite alopecia; however, the specimen was a generous excisional biopsy, allowing for a panoramic histologic view of the lesion. In the center of the specimen, hair follicles were absent, but residual follicular streamers and follicular remnants were surrounded by lymphocytic inflammation. Sebaceous glands were conspicuously absent, but foci with naked hairs, fibrosis, and granulomatous inflammation were seen. Peripherally, the hair follicles were thinned and miniaturized with an increased number of catagen/telogen hairs. Some follicles showed lamellar fibroplasia and perifollicular chronic inflammation. The inflammatory infiltrate consisted predominantly of helper T cells with a smaller population of B lymphocytes and a few plasma cells.6 In 2016, Lynch et al5 described a single case of tick bite alopecia and noted pseudolymphomatous inflammation with germinal center formation associated with hair miniaturization and an elevated catagen/telogen count; focal follicular mucinosis also was noted.Our histologic findings are similar to those of Castelli et al,6 except that the inflammatory infiltrate was clearly B-cell dominant, with a suggestion of germinal center formation, as noted by Lynch et al.5 This inflammatory pattern often can be encountered in a chronic tick bite lesion. Destruction of follicles and associated sebaceous glands and their replacement by follicular scars indicate that at least in the central portion of the lesion some permanent hair loss occurs. The presence of catagen/telogen hairs and miniaturized follicles indicates the potential for at least partial regrowth.
Similar to other investigators who have described tick bite alopecia, we can only speculate as to the mechanism by which clinical alopecia occurs. Given the density of the inflammatory infiltrate and perifollicular inflammation, it seems reasonable to assume that inflammation either destroys hair follicles or precipitates the catagen/telogen phase, resulting in temporary hair loss. The inflammation itself may be due to the presence of tick parts or the antigens in their saliva (or both). The delay between tick attachment and the onset of alopecia can be attributed to the time it takes follicles to cycle into the catagen/telogen phase and shed the hair shaft.
- Sauphar L. Alopecie peladoide consecutive a une piqure de tique. Bull Soc Fr Dermatol Syphiligr. 1921;28:442.
- Ross MS, Friede H. Alopecia due to tick bite. AMA Arch Derm. 1955;71:524-525.
- Heyl T. Tick bite alopecia. Clin Exp Dermatol. 1982;7:537-542.
- Marshall J. Alopecia after tick bite. S Afr Med J. 1966;40:555-556.
- Lynch MC, Milchak MA, Parnes H, et al. Tick bite alopecia: a report and review [published online April 19, 2016]. Am J Dermatopathol. doi:10.1097/DAD.0000000000000598.
- Castelli E, Caputo V, Morello V, et al. Local reactions to tick bites. Am J Dermatopathol. 2008;30:241-248.
- Nemeth NM, Ruder MG, Gerhold RW, et al. Demodectic mange, dermatophilosis, and other parasitic and bacterial dermatologic diseases in free-ranging white-tailed deer (Odocoileus virginianus) in the United States from 1975 to 2012. Vet Pathol. 2014;51:633-640.
- Welch DA, Samuel WM, Hudson RJ. Bioenergetic consequences of alopecia induced by Dermacentor albipictus (Acari: Ixodidae) on moose. J Med Entomol. 1990;27:656-660.
- Samuel WM. Locations of moose in northwestern Canada with hair loss probably caused by the winter tick, Dermacentor albipictus (Acari: Ixodidae). J Wildl Dis. 1989;25:436-439.
- Sauphar L. Alopecie peladoide consecutive a une piqure de tique. Bull Soc Fr Dermatol Syphiligr. 1921;28:442.
- Ross MS, Friede H. Alopecia due to tick bite. AMA Arch Derm. 1955;71:524-525.
- Heyl T. Tick bite alopecia. Clin Exp Dermatol. 1982;7:537-542.
- Marshall J. Alopecia after tick bite. S Afr Med J. 1966;40:555-556.
- Lynch MC, Milchak MA, Parnes H, et al. Tick bite alopecia: a report and review [published online April 19, 2016]. Am J Dermatopathol. doi:10.1097/DAD.0000000000000598.
- Castelli E, Caputo V, Morello V, et al. Local reactions to tick bites. Am J Dermatopathol. 2008;30:241-248.
- Nemeth NM, Ruder MG, Gerhold RW, et al. Demodectic mange, dermatophilosis, and other parasitic and bacterial dermatologic diseases in free-ranging white-tailed deer (Odocoileus virginianus) in the United States from 1975 to 2012. Vet Pathol. 2014;51:633-640.
- Welch DA, Samuel WM, Hudson RJ. Bioenergetic consequences of alopecia induced by Dermacentor albipictus (Acari: Ixodidae) on moose. J Med Entomol. 1990;27:656-660.
- Samuel WM. Locations of moose in northwestern Canada with hair loss probably caused by the winter tick, Dermacentor albipictus (Acari: Ixodidae). J Wildl Dis. 1989;25:436-439.
Practice Points
- Tick bite alopecia should be included in the differential diagnosis of both solitary and moth-eaten lesions of localized hair loss.
- In most cases, hair regrowth can be expected in a lesion of tick bite alopecia.
Subungual Exostosis
Case Report
A 41-year-old man with no dermatologic history presented for a skin examination. During a full-body skin examination, a lesion was identified on the right third toe that was partially visible underneath the nail plate. The patient stated that the lesion had been present for many years and did not appear to be growing but did cause occasional pain. On examination a 1-cm verrucous, hyperkeratotic, tan papule was noted at the distal end of the nail bed causing partial onycholysis (Figure 1). It was not tender to palpation.
A shave biopsy was obtained of the visible portion of the lesion, which revealed hyperkeratosis, acanthosis, and a population of dermal spindle cells in a myxoid stroma that could not be definitively identified. Special stains were nondiagnostic. The patient was referred to dermatologic surgery for rebiopsy of the lesion after removal of the nail plate. Mature bone was seen embedded in the dermis (Figure 2), and a diagnosis of subungual exostosis was made. Radiography of the digit confirmed a bony excrescence from the tuft of the toe, and the patient was referred to orthopedic surgery for definitive excision. There was no evidence of recurrence at 1-year follow-up.
Comment
Subungual exostosis is an uncommon benign bone tumor located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.1 Although it can occur on any digit, 70% to 80% of cases have arisen on the distal phalanx of the hallux.2 Both sexes are equally susceptible. The majority of lesions occur during the second or third decades of life and usually are asymptomatic unless there is trauma or infection. Growth of the lesion over time can cause lifting or deformity of the nail plate and can cause slight discomfort while walking if located on the great toe.3 Common differential diagnoses include osteochondroma, wart, fibroma, paronychia, myositis ossificans, and pyogenic granuloma.3,4 Diagnosis can be confirmed with radiography, which should be performed prior to any biopsy or invasive procedure. In our patient, initial radiography could have obviated the need for 2 biopsies prior to definitive excision. Histopathologic evaluation typically reveals mature trabecular bone (Figure 2) surrounded by a fibrocartilage cap.
Subungual exostosis begins as an area of proliferating fibrous tissue with cartilaginous metaplasia located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.1 This cartilage undergoes enchondral ossification and is converted to trabecular bone. As the lesion grows and matures, the cartilaginous cap blends imperceptibly with the nail bed and comes into continuity with the underlying distal phalanx.1,3 This process continues until the lesion fuses completely with the distal phalanx.1 Although the cause of subungual exostosis has not been clearly established, chronic irritation, trauma, and chronic infections are considered causative factors of fibrocartilaginous metaplasia.4
The most commonly accepted treatment of subungual exostosis is a localized excision. Partial or total removal of the nail has traditionally be advocated to ensure complete excision of the exostosis, a nail-sparing technique that has been shown to enhance cosmetic results.3 Incomplete excision and incomplete maturation of the lesion have been reported to be responsible for almost 50% of recurrences.3 This high recurrence rate is due to difficulty in ensuring a total excision because the gradual merging of the fibrocartilage cap with the overlying nail bed makes it impossible to develop a cleavage plane5; as a result, it has been suggested that excision should only be attempted after maturation of the tumor so the cleavage plane can fully develop. Other studies claim that delaying treatment can result in elevation and deformity of the nail, pain, and secondary periungual infection.3
Conclusion
Subungual exostosis is a benign bony tumor of the distal phalanx that can cause pain and onycholysis. Radiography of the affected digit is a noninvasive way to confirm the diagnosis and should be part of the initial workup of any suspicious subungual tumor. Once identified, complete removal of the exostosis by excision has been shown to be an effective treatment with few complications.
- Letts M, Davidson D, Nizalik E. Subungual exostosis: diagnosis and treatment in children. J Trauma. 1998;44:346-349.
- Starnes A, Crosby K, Rowe DJ, et al. Subungual exostosis: a simple surgical technique. Dermatol Surg. 2012;38:258-260.
- Lokiec F, Ezra E, Krasin E, et al. A simple and efficient surgical technique for subungual exostosis. J Pediatr Orthop. 2001;21:76-79.
- Turan H, Uslu M, Erdem H. A case of subungual exostosis. Indian J Dermatol Venereol Leprol. 2012;78:186.
- Miller-Breslow A, Dorfman HD. Dupuytren’s (subungual) exostosis. Am J Surg Pathol. 1988;12:368-378.
Case Report
A 41-year-old man with no dermatologic history presented for a skin examination. During a full-body skin examination, a lesion was identified on the right third toe that was partially visible underneath the nail plate. The patient stated that the lesion had been present for many years and did not appear to be growing but did cause occasional pain. On examination a 1-cm verrucous, hyperkeratotic, tan papule was noted at the distal end of the nail bed causing partial onycholysis (Figure 1). It was not tender to palpation.
A shave biopsy was obtained of the visible portion of the lesion, which revealed hyperkeratosis, acanthosis, and a population of dermal spindle cells in a myxoid stroma that could not be definitively identified. Special stains were nondiagnostic. The patient was referred to dermatologic surgery for rebiopsy of the lesion after removal of the nail plate. Mature bone was seen embedded in the dermis (Figure 2), and a diagnosis of subungual exostosis was made. Radiography of the digit confirmed a bony excrescence from the tuft of the toe, and the patient was referred to orthopedic surgery for definitive excision. There was no evidence of recurrence at 1-year follow-up.
Comment
Subungual exostosis is an uncommon benign bone tumor located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.1 Although it can occur on any digit, 70% to 80% of cases have arisen on the distal phalanx of the hallux.2 Both sexes are equally susceptible. The majority of lesions occur during the second or third decades of life and usually are asymptomatic unless there is trauma or infection. Growth of the lesion over time can cause lifting or deformity of the nail plate and can cause slight discomfort while walking if located on the great toe.3 Common differential diagnoses include osteochondroma, wart, fibroma, paronychia, myositis ossificans, and pyogenic granuloma.3,4 Diagnosis can be confirmed with radiography, which should be performed prior to any biopsy or invasive procedure. In our patient, initial radiography could have obviated the need for 2 biopsies prior to definitive excision. Histopathologic evaluation typically reveals mature trabecular bone (Figure 2) surrounded by a fibrocartilage cap.
Subungual exostosis begins as an area of proliferating fibrous tissue with cartilaginous metaplasia located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.1 This cartilage undergoes enchondral ossification and is converted to trabecular bone. As the lesion grows and matures, the cartilaginous cap blends imperceptibly with the nail bed and comes into continuity with the underlying distal phalanx.1,3 This process continues until the lesion fuses completely with the distal phalanx.1 Although the cause of subungual exostosis has not been clearly established, chronic irritation, trauma, and chronic infections are considered causative factors of fibrocartilaginous metaplasia.4
The most commonly accepted treatment of subungual exostosis is a localized excision. Partial or total removal of the nail has traditionally be advocated to ensure complete excision of the exostosis, a nail-sparing technique that has been shown to enhance cosmetic results.3 Incomplete excision and incomplete maturation of the lesion have been reported to be responsible for almost 50% of recurrences.3 This high recurrence rate is due to difficulty in ensuring a total excision because the gradual merging of the fibrocartilage cap with the overlying nail bed makes it impossible to develop a cleavage plane5; as a result, it has been suggested that excision should only be attempted after maturation of the tumor so the cleavage plane can fully develop. Other studies claim that delaying treatment can result in elevation and deformity of the nail, pain, and secondary periungual infection.3
Conclusion
Subungual exostosis is a benign bony tumor of the distal phalanx that can cause pain and onycholysis. Radiography of the affected digit is a noninvasive way to confirm the diagnosis and should be part of the initial workup of any suspicious subungual tumor. Once identified, complete removal of the exostosis by excision has been shown to be an effective treatment with few complications.
Case Report
A 41-year-old man with no dermatologic history presented for a skin examination. During a full-body skin examination, a lesion was identified on the right third toe that was partially visible underneath the nail plate. The patient stated that the lesion had been present for many years and did not appear to be growing but did cause occasional pain. On examination a 1-cm verrucous, hyperkeratotic, tan papule was noted at the distal end of the nail bed causing partial onycholysis (Figure 1). It was not tender to palpation.
A shave biopsy was obtained of the visible portion of the lesion, which revealed hyperkeratosis, acanthosis, and a population of dermal spindle cells in a myxoid stroma that could not be definitively identified. Special stains were nondiagnostic. The patient was referred to dermatologic surgery for rebiopsy of the lesion after removal of the nail plate. Mature bone was seen embedded in the dermis (Figure 2), and a diagnosis of subungual exostosis was made. Radiography of the digit confirmed a bony excrescence from the tuft of the toe, and the patient was referred to orthopedic surgery for definitive excision. There was no evidence of recurrence at 1-year follow-up.
Comment
Subungual exostosis is an uncommon benign bone tumor located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.1 Although it can occur on any digit, 70% to 80% of cases have arisen on the distal phalanx of the hallux.2 Both sexes are equally susceptible. The majority of lesions occur during the second or third decades of life and usually are asymptomatic unless there is trauma or infection. Growth of the lesion over time can cause lifting or deformity of the nail plate and can cause slight discomfort while walking if located on the great toe.3 Common differential diagnoses include osteochondroma, wart, fibroma, paronychia, myositis ossificans, and pyogenic granuloma.3,4 Diagnosis can be confirmed with radiography, which should be performed prior to any biopsy or invasive procedure. In our patient, initial radiography could have obviated the need for 2 biopsies prior to definitive excision. Histopathologic evaluation typically reveals mature trabecular bone (Figure 2) surrounded by a fibrocartilage cap.
Subungual exostosis begins as an area of proliferating fibrous tissue with cartilaginous metaplasia located beneath or adjacent to the nail bed on the dorsal aspect of the distal phalanx.1 This cartilage undergoes enchondral ossification and is converted to trabecular bone. As the lesion grows and matures, the cartilaginous cap blends imperceptibly with the nail bed and comes into continuity with the underlying distal phalanx.1,3 This process continues until the lesion fuses completely with the distal phalanx.1 Although the cause of subungual exostosis has not been clearly established, chronic irritation, trauma, and chronic infections are considered causative factors of fibrocartilaginous metaplasia.4
The most commonly accepted treatment of subungual exostosis is a localized excision. Partial or total removal of the nail has traditionally be advocated to ensure complete excision of the exostosis, a nail-sparing technique that has been shown to enhance cosmetic results.3 Incomplete excision and incomplete maturation of the lesion have been reported to be responsible for almost 50% of recurrences.3 This high recurrence rate is due to difficulty in ensuring a total excision because the gradual merging of the fibrocartilage cap with the overlying nail bed makes it impossible to develop a cleavage plane5; as a result, it has been suggested that excision should only be attempted after maturation of the tumor so the cleavage plane can fully develop. Other studies claim that delaying treatment can result in elevation and deformity of the nail, pain, and secondary periungual infection.3
Conclusion
Subungual exostosis is a benign bony tumor of the distal phalanx that can cause pain and onycholysis. Radiography of the affected digit is a noninvasive way to confirm the diagnosis and should be part of the initial workup of any suspicious subungual tumor. Once identified, complete removal of the exostosis by excision has been shown to be an effective treatment with few complications.
- Letts M, Davidson D, Nizalik E. Subungual exostosis: diagnosis and treatment in children. J Trauma. 1998;44:346-349.
- Starnes A, Crosby K, Rowe DJ, et al. Subungual exostosis: a simple surgical technique. Dermatol Surg. 2012;38:258-260.
- Lokiec F, Ezra E, Krasin E, et al. A simple and efficient surgical technique for subungual exostosis. J Pediatr Orthop. 2001;21:76-79.
- Turan H, Uslu M, Erdem H. A case of subungual exostosis. Indian J Dermatol Venereol Leprol. 2012;78:186.
- Miller-Breslow A, Dorfman HD. Dupuytren’s (subungual) exostosis. Am J Surg Pathol. 1988;12:368-378.
- Letts M, Davidson D, Nizalik E. Subungual exostosis: diagnosis and treatment in children. J Trauma. 1998;44:346-349.
- Starnes A, Crosby K, Rowe DJ, et al. Subungual exostosis: a simple surgical technique. Dermatol Surg. 2012;38:258-260.
- Lokiec F, Ezra E, Krasin E, et al. A simple and efficient surgical technique for subungual exostosis. J Pediatr Orthop. 2001;21:76-79.
- Turan H, Uslu M, Erdem H. A case of subungual exostosis. Indian J Dermatol Venereol Leprol. 2012;78:186.
- Miller-Breslow A, Dorfman HD. Dupuytren’s (subungual) exostosis. Am J Surg Pathol. 1988;12:368-378.
Practice Points
- Subungual exostosis is a benign tumor that is most common on the hallux.
- Plain radiographs can identify an exostosis and should be part of the initial workup of any subungual tumor.
- Surgical excision is an effective and well-tolerated treatment of subungual exostosis.
Fingernail Photo-onycholysis After Aminolevulinic Acid–Photodynamic Therapy Under Blue Light for Treatment of Actinic Keratoses on the Face
To the Editor:
Topical photodynamic therapy (PDT) is one of several effective treatments of actinic keratoses (AKs). Photodynamic therapy involves selection of a lesion field, application of a photosensitizer drug, incubation for an explicit period of time, and illumination of the area from a light source corresponding to the absorption spectrum of the chosen drug.1 A photosensitizer drug used in PDT to target AK is aminolevulinic acid (ALA). Aminolevulinic acid converts disease tissue to photoactivatable porphyrins, especially protoporphyrin IX, which has its largest absorption peak (410 nm) in the blue spectrum, with smaller absorption peaks at 505, 540, 580, and 630 nm. Photodynamic therapy treatments historically have been carried out under red light (peak emissions, 630 nm) to improve tissue penetration, which is superior in efficacy when treating Bowen disease and basal cell carcinoma.1,2 Broadband blue light (peak emission, 417 nm) now is routinely used and has been proven effective in combination with ALA for the treatment of AK.3 It was approved by the US Food and Drug Administration for AKs in 1999.4
Photo-onycholysis is a photosensitivity reaction defined as separation of the nail plate from the nail bed. There are 4 different types of photo-onycholysis characterized by appearance and by the number of digits affected: Type I is denoted by the involvement of several fingers, with half-moon–shaped separations of the nail plate. Type II affects a single finger and corresponds to a brown, defined, circular notch opening distally. Type III, which involves several fingers, is defined as round yellow stains in the central portion of the nail that turn red after 5 to 10 days. Type IV has been associated with bullae under the nails.5 There have been cases of photo-onycholysis arising after exposure to UV light following ingestion of certain prescription drugs or spontaneously,6 and a single case following PDT to the hands with red light.5 We report a case of fingernail photo-onycholysis resulting from ALA-PDT for the treatment of perioral AK.
A 65-year-old woman was treated for AKs on the perioral region of the face with PDT. Aminolevulinic acid hydrochloride 20% was applied to the lips and allowed to incubate for 60 minutes. Her face was illuminated with 10 J/cm² of blue light (417 nm) for 16 minutes and 40 seconds. Sunscreen (sun protection factor 40) was applied to the area immediately after treatment, and the patient was thoroughly counseled to avoid sunlight for the next 48 hours and to use sun protection. Within 72 hours following treatment, the patient reported all 10 fingernails noticeably separated from the nail bed with minimal pain, corresponding to type I photo-onycholysis (Figure). The patient’s only medications were vitamin D (1000 mg once daily) and calcium supplements (1500 mg twice daily). Although the patient exercised strict UV light avoidance for the face, her hands were not protected when she went gardening directly after the treatment. At 5 weeks, the patient returned for her second ALA-PDT treatment of perioral AK and a fungal culture was taken of the left third fingernail, which returned negative results. Poly-ureaurethane nail lacquer 16% was prescribed and was used once daily to protect and strengthen the fingernails. The patient returned for follow-up in clinic after 13 weeks and photo-onycholysis was resolving. Photo-onycholysis is categorized as a phototoxic reaction often associated with drug intake, more specifically with the use of tetracyclines, psoralens, and fluoroquinolones; less commonly with oral contraceptives; or spontaneously.6 It usually is recognized as a crescent-shaped distal separation of the nail surrounded by pigment. The action spectrum is believed to include UVA and UVB, though the exact mechanisms have not been confirmed.5
Our case provides evidence for risks involving the development of photo-onycholysis following PDT. We have no reason to believe there was systemic absorption of ALA, as there were no visible vesicles on the arms or hands after the treatment. Negative fungal culture results excluded onychomycosis. It is our hypothesis that the patient touched her face with her fingernails during the 60-minute incubation time prior to ALA-PDT treatment under blue light, inadvertently collecting ALA under the fingernails. Once she exposed her hands to sunlight while gardening after treatment, the nails likely reacted with the ALA in response to the UV radiation, thus triggering photo-onycholysis.
This case represents a report of fingernail photo-onycholysis from ALA-PDT under blue light as well as a report following treatment of AK not located on the hands with PDT. Although the photo-onycholysis did resolve within a few months of treatment, our case demonstrates the importance of counseling patients more specifically about isolating the ALA treatment zone from nontreated areas on the body during incubation. Improper UV light protection following ALA-PDT is known to produce phototoxic reactions and our case supports this outcome.
- Morton CA, McKenna KE, Rhodes LE. Guidelines for topical photodynamic therapy: update. Br J Dermatol. 2008;159:1245-1266.
- Hauschild A. Photodynamic therapy for actinic keratoses: procedure matters? Br J Dermatol. 2012;166:3-5.
- Alexiades-Armenakas M. Laser-mediated photodynamic therapy. Clin Dermatol. 2006;24:16-25.
- Babilas P, Schreml S, Landthaler M, et al. Photodynamic therapy in dermatology: state-of-the-art. Photodermatol Photoimmunol Photomed. 2010;26:118-132.
- Hanneken S, Wessendorf U, Neumann NJ. Photodynamic onycholysis: first report of photo-onycholysis after photodynamic therapy. Clin Exp Dermatol. 2008;33:659-660.
- Baran R, Juhlin L. Photoonycholysis. Photodermatol Photoimmunol Photomed. 2002;18:202-207.
To the Editor:
Topical photodynamic therapy (PDT) is one of several effective treatments of actinic keratoses (AKs). Photodynamic therapy involves selection of a lesion field, application of a photosensitizer drug, incubation for an explicit period of time, and illumination of the area from a light source corresponding to the absorption spectrum of the chosen drug.1 A photosensitizer drug used in PDT to target AK is aminolevulinic acid (ALA). Aminolevulinic acid converts disease tissue to photoactivatable porphyrins, especially protoporphyrin IX, which has its largest absorption peak (410 nm) in the blue spectrum, with smaller absorption peaks at 505, 540, 580, and 630 nm. Photodynamic therapy treatments historically have been carried out under red light (peak emissions, 630 nm) to improve tissue penetration, which is superior in efficacy when treating Bowen disease and basal cell carcinoma.1,2 Broadband blue light (peak emission, 417 nm) now is routinely used and has been proven effective in combination with ALA for the treatment of AK.3 It was approved by the US Food and Drug Administration for AKs in 1999.4
Photo-onycholysis is a photosensitivity reaction defined as separation of the nail plate from the nail bed. There are 4 different types of photo-onycholysis characterized by appearance and by the number of digits affected: Type I is denoted by the involvement of several fingers, with half-moon–shaped separations of the nail plate. Type II affects a single finger and corresponds to a brown, defined, circular notch opening distally. Type III, which involves several fingers, is defined as round yellow stains in the central portion of the nail that turn red after 5 to 10 days. Type IV has been associated with bullae under the nails.5 There have been cases of photo-onycholysis arising after exposure to UV light following ingestion of certain prescription drugs or spontaneously,6 and a single case following PDT to the hands with red light.5 We report a case of fingernail photo-onycholysis resulting from ALA-PDT for the treatment of perioral AK.
A 65-year-old woman was treated for AKs on the perioral region of the face with PDT. Aminolevulinic acid hydrochloride 20% was applied to the lips and allowed to incubate for 60 minutes. Her face was illuminated with 10 J/cm² of blue light (417 nm) for 16 minutes and 40 seconds. Sunscreen (sun protection factor 40) was applied to the area immediately after treatment, and the patient was thoroughly counseled to avoid sunlight for the next 48 hours and to use sun protection. Within 72 hours following treatment, the patient reported all 10 fingernails noticeably separated from the nail bed with minimal pain, corresponding to type I photo-onycholysis (Figure). The patient’s only medications were vitamin D (1000 mg once daily) and calcium supplements (1500 mg twice daily). Although the patient exercised strict UV light avoidance for the face, her hands were not protected when she went gardening directly after the treatment. At 5 weeks, the patient returned for her second ALA-PDT treatment of perioral AK and a fungal culture was taken of the left third fingernail, which returned negative results. Poly-ureaurethane nail lacquer 16% was prescribed and was used once daily to protect and strengthen the fingernails. The patient returned for follow-up in clinic after 13 weeks and photo-onycholysis was resolving. Photo-onycholysis is categorized as a phototoxic reaction often associated with drug intake, more specifically with the use of tetracyclines, psoralens, and fluoroquinolones; less commonly with oral contraceptives; or spontaneously.6 It usually is recognized as a crescent-shaped distal separation of the nail surrounded by pigment. The action spectrum is believed to include UVA and UVB, though the exact mechanisms have not been confirmed.5
Our case provides evidence for risks involving the development of photo-onycholysis following PDT. We have no reason to believe there was systemic absorption of ALA, as there were no visible vesicles on the arms or hands after the treatment. Negative fungal culture results excluded onychomycosis. It is our hypothesis that the patient touched her face with her fingernails during the 60-minute incubation time prior to ALA-PDT treatment under blue light, inadvertently collecting ALA under the fingernails. Once she exposed her hands to sunlight while gardening after treatment, the nails likely reacted with the ALA in response to the UV radiation, thus triggering photo-onycholysis.
This case represents a report of fingernail photo-onycholysis from ALA-PDT under blue light as well as a report following treatment of AK not located on the hands with PDT. Although the photo-onycholysis did resolve within a few months of treatment, our case demonstrates the importance of counseling patients more specifically about isolating the ALA treatment zone from nontreated areas on the body during incubation. Improper UV light protection following ALA-PDT is known to produce phototoxic reactions and our case supports this outcome.
To the Editor:
Topical photodynamic therapy (PDT) is one of several effective treatments of actinic keratoses (AKs). Photodynamic therapy involves selection of a lesion field, application of a photosensitizer drug, incubation for an explicit period of time, and illumination of the area from a light source corresponding to the absorption spectrum of the chosen drug.1 A photosensitizer drug used in PDT to target AK is aminolevulinic acid (ALA). Aminolevulinic acid converts disease tissue to photoactivatable porphyrins, especially protoporphyrin IX, which has its largest absorption peak (410 nm) in the blue spectrum, with smaller absorption peaks at 505, 540, 580, and 630 nm. Photodynamic therapy treatments historically have been carried out under red light (peak emissions, 630 nm) to improve tissue penetration, which is superior in efficacy when treating Bowen disease and basal cell carcinoma.1,2 Broadband blue light (peak emission, 417 nm) now is routinely used and has been proven effective in combination with ALA for the treatment of AK.3 It was approved by the US Food and Drug Administration for AKs in 1999.4
Photo-onycholysis is a photosensitivity reaction defined as separation of the nail plate from the nail bed. There are 4 different types of photo-onycholysis characterized by appearance and by the number of digits affected: Type I is denoted by the involvement of several fingers, with half-moon–shaped separations of the nail plate. Type II affects a single finger and corresponds to a brown, defined, circular notch opening distally. Type III, which involves several fingers, is defined as round yellow stains in the central portion of the nail that turn red after 5 to 10 days. Type IV has been associated with bullae under the nails.5 There have been cases of photo-onycholysis arising after exposure to UV light following ingestion of certain prescription drugs or spontaneously,6 and a single case following PDT to the hands with red light.5 We report a case of fingernail photo-onycholysis resulting from ALA-PDT for the treatment of perioral AK.
A 65-year-old woman was treated for AKs on the perioral region of the face with PDT. Aminolevulinic acid hydrochloride 20% was applied to the lips and allowed to incubate for 60 minutes. Her face was illuminated with 10 J/cm² of blue light (417 nm) for 16 minutes and 40 seconds. Sunscreen (sun protection factor 40) was applied to the area immediately after treatment, and the patient was thoroughly counseled to avoid sunlight for the next 48 hours and to use sun protection. Within 72 hours following treatment, the patient reported all 10 fingernails noticeably separated from the nail bed with minimal pain, corresponding to type I photo-onycholysis (Figure). The patient’s only medications were vitamin D (1000 mg once daily) and calcium supplements (1500 mg twice daily). Although the patient exercised strict UV light avoidance for the face, her hands were not protected when she went gardening directly after the treatment. At 5 weeks, the patient returned for her second ALA-PDT treatment of perioral AK and a fungal culture was taken of the left third fingernail, which returned negative results. Poly-ureaurethane nail lacquer 16% was prescribed and was used once daily to protect and strengthen the fingernails. The patient returned for follow-up in clinic after 13 weeks and photo-onycholysis was resolving. Photo-onycholysis is categorized as a phototoxic reaction often associated with drug intake, more specifically with the use of tetracyclines, psoralens, and fluoroquinolones; less commonly with oral contraceptives; or spontaneously.6 It usually is recognized as a crescent-shaped distal separation of the nail surrounded by pigment. The action spectrum is believed to include UVA and UVB, though the exact mechanisms have not been confirmed.5
Our case provides evidence for risks involving the development of photo-onycholysis following PDT. We have no reason to believe there was systemic absorption of ALA, as there were no visible vesicles on the arms or hands after the treatment. Negative fungal culture results excluded onychomycosis. It is our hypothesis that the patient touched her face with her fingernails during the 60-minute incubation time prior to ALA-PDT treatment under blue light, inadvertently collecting ALA under the fingernails. Once she exposed her hands to sunlight while gardening after treatment, the nails likely reacted with the ALA in response to the UV radiation, thus triggering photo-onycholysis.
This case represents a report of fingernail photo-onycholysis from ALA-PDT under blue light as well as a report following treatment of AK not located on the hands with PDT. Although the photo-onycholysis did resolve within a few months of treatment, our case demonstrates the importance of counseling patients more specifically about isolating the ALA treatment zone from nontreated areas on the body during incubation. Improper UV light protection following ALA-PDT is known to produce phototoxic reactions and our case supports this outcome.
- Morton CA, McKenna KE, Rhodes LE. Guidelines for topical photodynamic therapy: update. Br J Dermatol. 2008;159:1245-1266.
- Hauschild A. Photodynamic therapy for actinic keratoses: procedure matters? Br J Dermatol. 2012;166:3-5.
- Alexiades-Armenakas M. Laser-mediated photodynamic therapy. Clin Dermatol. 2006;24:16-25.
- Babilas P, Schreml S, Landthaler M, et al. Photodynamic therapy in dermatology: state-of-the-art. Photodermatol Photoimmunol Photomed. 2010;26:118-132.
- Hanneken S, Wessendorf U, Neumann NJ. Photodynamic onycholysis: first report of photo-onycholysis after photodynamic therapy. Clin Exp Dermatol. 2008;33:659-660.
- Baran R, Juhlin L. Photoonycholysis. Photodermatol Photoimmunol Photomed. 2002;18:202-207.
- Morton CA, McKenna KE, Rhodes LE. Guidelines for topical photodynamic therapy: update. Br J Dermatol. 2008;159:1245-1266.
- Hauschild A. Photodynamic therapy for actinic keratoses: procedure matters? Br J Dermatol. 2012;166:3-5.
- Alexiades-Armenakas M. Laser-mediated photodynamic therapy. Clin Dermatol. 2006;24:16-25.
- Babilas P, Schreml S, Landthaler M, et al. Photodynamic therapy in dermatology: state-of-the-art. Photodermatol Photoimmunol Photomed. 2010;26:118-132.
- Hanneken S, Wessendorf U, Neumann NJ. Photodynamic onycholysis: first report of photo-onycholysis after photodynamic therapy. Clin Exp Dermatol. 2008;33:659-660.
- Baran R, Juhlin L. Photoonycholysis. Photodermatol Photoimmunol Photomed. 2002;18:202-207.
Practice Points
- Photodynamic therapy with aminolevulinic acid (ALA) is an effective treatment of actinic keratoses but can produce unexpected side effects in locations distant from initial therapy sites.
- It is important to counsel patients prior to initiating photodynamic therapy with ALA about isolating the ALA treatment zone from nontreated areas on the body during incubation.
Studies highlight diagnostic and treatment challenges in hidradenitis suppurativa
SCOTTSDALE, ARIZ. – Patients with hidradenitis suppurativa (HS) may be misdiagnosed when they see providers who are not dermatologists – as is usually the case during the initial years of their disease, according to a large analysis of medical claims data.
The findings highlight the need for visual diagnostic aids and specific guidelines for treating HS that target nondermatologists, Melissa Butt, MPH, of Penn State Hershey (Pa.) Medical Center, said during an interview at the annual meeting of the Society for Investigative Dermatology. She presented the findings during a poster session at the meeting.
HS is a chronic inflammatory disease of the hair follicles that affects 0.5%-4% of people in the United States. In past studies, up to 12 years elapsed between disease onset and diagnosis, in part because patients often cannot readily access dermatologists, Ms. Butt said. To better understand patterns of health care use during the years leading up to HS diagnosis, she and her colleagues used MarketScan data to identify 1,733 patients with HS-specific medical care claims filed in 2012 and 2013. Then they looked back at medical claims for these patients during 2008 through 2011, before the patients were diagnosed with HS. The cohort averaged 37 years of age (standard deviation, 15 years), and 73% were female.
Among 239,892 claims filed before patients were diagnosed with HS, 11,381 (4.7%) included codes for other diseases of the skin and subcutaneous tissues, Ms. Butt said. Dermatologists filed only 31% of these skin-specific claims, while 69% were filed by other providers, such as family practitioners, internists, emergency department physicians, and acute care hospitalists.
Notably, about two-thirds of the skin-specific diagnostic codes could have represented a misdiagnosis of HS. These codes included conditions such as abscesses, carbuncles, local infections, ulcers, and diseases of the sebaceous glands.
The fact that 78% of visits occurred in offices and other outpatient settings further underscores the need to improve the detection and care of HS in these environments, Ms. Butt said. Given current national shortages of dermatologists, visual HS diagnostic aids and “detailed, multistep clinical practice guidelines” for nondermatologists could help improve care of HS while patients wait to see the specialists, she added.
A second poster presented at the meeting provided results of a study on the use and impact of antibiotics in the treatment of HS. Alexander Fischer of Johns Hopkins University, Baltimore, and his associates studied antibiotic prescriptions and bacterial cultures from the lesions of 239 patients with HS who were treated at Johns Hopkins medical facilities between 2010 and 2015. Not only were 51% of HS patients on antibiotics at the time of culture, but these patients’ lesions were significantly more likely to contain antibiotic-resistant bacteria than were those of patients not on antibiotics.
Strikingly, Proteus species were isolated from nearly half of patients on trimethoprim-sulfamethoxazole (TMP/SMX), and 88% of colonies were resistant to TMP/SMX, while only 13% of cultures from untreated patients grew Proteus (P less than .001) and all were TMP/SMX-susceptible (P less than .001). Likewise, 100% of methicillin-resistant Staphylococcus aureus (MRSA) strains from patients prescribed ciprofloxacin were resistant to it, compared with a 10% background rate of ciprofloxacin resistance among MRSA from patients not taking antibiotics (P = .04). In addition, the proportion of other S. aureus strains that were clindamycin-resistant was higher when patients were taking this antibiotic than when they were not (63% versus 17%; P = .03).
The results “raise questions” about whether antibiotics should be used in HS patients who are not clearly benefiting from them, according to the researchers.
The authors of both studies reported no funding sources and had no disclosures.
SCOTTSDALE, ARIZ. – Patients with hidradenitis suppurativa (HS) may be misdiagnosed when they see providers who are not dermatologists – as is usually the case during the initial years of their disease, according to a large analysis of medical claims data.
The findings highlight the need for visual diagnostic aids and specific guidelines for treating HS that target nondermatologists, Melissa Butt, MPH, of Penn State Hershey (Pa.) Medical Center, said during an interview at the annual meeting of the Society for Investigative Dermatology. She presented the findings during a poster session at the meeting.
HS is a chronic inflammatory disease of the hair follicles that affects 0.5%-4% of people in the United States. In past studies, up to 12 years elapsed between disease onset and diagnosis, in part because patients often cannot readily access dermatologists, Ms. Butt said. To better understand patterns of health care use during the years leading up to HS diagnosis, she and her colleagues used MarketScan data to identify 1,733 patients with HS-specific medical care claims filed in 2012 and 2013. Then they looked back at medical claims for these patients during 2008 through 2011, before the patients were diagnosed with HS. The cohort averaged 37 years of age (standard deviation, 15 years), and 73% were female.
Among 239,892 claims filed before patients were diagnosed with HS, 11,381 (4.7%) included codes for other diseases of the skin and subcutaneous tissues, Ms. Butt said. Dermatologists filed only 31% of these skin-specific claims, while 69% were filed by other providers, such as family practitioners, internists, emergency department physicians, and acute care hospitalists.
Notably, about two-thirds of the skin-specific diagnostic codes could have represented a misdiagnosis of HS. These codes included conditions such as abscesses, carbuncles, local infections, ulcers, and diseases of the sebaceous glands.
The fact that 78% of visits occurred in offices and other outpatient settings further underscores the need to improve the detection and care of HS in these environments, Ms. Butt said. Given current national shortages of dermatologists, visual HS diagnostic aids and “detailed, multistep clinical practice guidelines” for nondermatologists could help improve care of HS while patients wait to see the specialists, she added.
A second poster presented at the meeting provided results of a study on the use and impact of antibiotics in the treatment of HS. Alexander Fischer of Johns Hopkins University, Baltimore, and his associates studied antibiotic prescriptions and bacterial cultures from the lesions of 239 patients with HS who were treated at Johns Hopkins medical facilities between 2010 and 2015. Not only were 51% of HS patients on antibiotics at the time of culture, but these patients’ lesions were significantly more likely to contain antibiotic-resistant bacteria than were those of patients not on antibiotics.
Strikingly, Proteus species were isolated from nearly half of patients on trimethoprim-sulfamethoxazole (TMP/SMX), and 88% of colonies were resistant to TMP/SMX, while only 13% of cultures from untreated patients grew Proteus (P less than .001) and all were TMP/SMX-susceptible (P less than .001). Likewise, 100% of methicillin-resistant Staphylococcus aureus (MRSA) strains from patients prescribed ciprofloxacin were resistant to it, compared with a 10% background rate of ciprofloxacin resistance among MRSA from patients not taking antibiotics (P = .04). In addition, the proportion of other S. aureus strains that were clindamycin-resistant was higher when patients were taking this antibiotic than when they were not (63% versus 17%; P = .03).
The results “raise questions” about whether antibiotics should be used in HS patients who are not clearly benefiting from them, according to the researchers.
The authors of both studies reported no funding sources and had no disclosures.
SCOTTSDALE, ARIZ. – Patients with hidradenitis suppurativa (HS) may be misdiagnosed when they see providers who are not dermatologists – as is usually the case during the initial years of their disease, according to a large analysis of medical claims data.
The findings highlight the need for visual diagnostic aids and specific guidelines for treating HS that target nondermatologists, Melissa Butt, MPH, of Penn State Hershey (Pa.) Medical Center, said during an interview at the annual meeting of the Society for Investigative Dermatology. She presented the findings during a poster session at the meeting.
HS is a chronic inflammatory disease of the hair follicles that affects 0.5%-4% of people in the United States. In past studies, up to 12 years elapsed between disease onset and diagnosis, in part because patients often cannot readily access dermatologists, Ms. Butt said. To better understand patterns of health care use during the years leading up to HS diagnosis, she and her colleagues used MarketScan data to identify 1,733 patients with HS-specific medical care claims filed in 2012 and 2013. Then they looked back at medical claims for these patients during 2008 through 2011, before the patients were diagnosed with HS. The cohort averaged 37 years of age (standard deviation, 15 years), and 73% were female.
Among 239,892 claims filed before patients were diagnosed with HS, 11,381 (4.7%) included codes for other diseases of the skin and subcutaneous tissues, Ms. Butt said. Dermatologists filed only 31% of these skin-specific claims, while 69% were filed by other providers, such as family practitioners, internists, emergency department physicians, and acute care hospitalists.
Notably, about two-thirds of the skin-specific diagnostic codes could have represented a misdiagnosis of HS. These codes included conditions such as abscesses, carbuncles, local infections, ulcers, and diseases of the sebaceous glands.
The fact that 78% of visits occurred in offices and other outpatient settings further underscores the need to improve the detection and care of HS in these environments, Ms. Butt said. Given current national shortages of dermatologists, visual HS diagnostic aids and “detailed, multistep clinical practice guidelines” for nondermatologists could help improve care of HS while patients wait to see the specialists, she added.
A second poster presented at the meeting provided results of a study on the use and impact of antibiotics in the treatment of HS. Alexander Fischer of Johns Hopkins University, Baltimore, and his associates studied antibiotic prescriptions and bacterial cultures from the lesions of 239 patients with HS who were treated at Johns Hopkins medical facilities between 2010 and 2015. Not only were 51% of HS patients on antibiotics at the time of culture, but these patients’ lesions were significantly more likely to contain antibiotic-resistant bacteria than were those of patients not on antibiotics.
Strikingly, Proteus species were isolated from nearly half of patients on trimethoprim-sulfamethoxazole (TMP/SMX), and 88% of colonies were resistant to TMP/SMX, while only 13% of cultures from untreated patients grew Proteus (P less than .001) and all were TMP/SMX-susceptible (P less than .001). Likewise, 100% of methicillin-resistant Staphylococcus aureus (MRSA) strains from patients prescribed ciprofloxacin were resistant to it, compared with a 10% background rate of ciprofloxacin resistance among MRSA from patients not taking antibiotics (P = .04). In addition, the proportion of other S. aureus strains that were clindamycin-resistant was higher when patients were taking this antibiotic than when they were not (63% versus 17%; P = .03).
The results “raise questions” about whether antibiotics should be used in HS patients who are not clearly benefiting from them, according to the researchers.
The authors of both studies reported no funding sources and had no disclosures.
AT THE 2016 SID ANNUAL MEETING
Key clinical point: Two studies underscored current challenges in diagnosing and treating hidradenitis suppurativa (HS).
Major finding: HS was usually diagnosed in outpatient settings by nondermatologists who often initially filed claims for carbuncles, ulcers, and other conditions that are confused with HS. In a separate study, antibiotic-resistant bacteria were significantly more prevalent in the lesions of HS patients who were receiving antibiotics than in patients who were not taking antibiotics.
Data source: A medical claims analysis of 1,733 patients with HS, and a study of antibiotic prescriptions and bacterial cultures from 239 patients with HS.
Disclosures: The authors of both studies reported no funding sources and had no disclosures.
