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Laboratory work revealed a normal CBC and differential, an elevated C-reactive protein (CRP) and sedimentation rate (ESR), negative antistreptolysin O (ASO) titers, negative pregnancy test, a normal urinalysis, and negative blood, throat, and urine cultures. A chest x-ray also was negative as well as angiotensin-converting enzyme (ACE) levels. Tuberculosis interferon-gamma release essay was negative.

The patient was diagnosed with erythema nodosum (EN), based on physical exam and history of the lesions. In her particular case, infectious causes including streptococcus infection, tuberculosis, and coccidioidomycosis were ruled out. There were no x-ray findings that suggested sarcoidosis and her ACE level was within normal limits. The pregnancy test also was negative. Given her recent start on OCs, this was thought to be the cause of the lesions.

She was treated with elevation, compression stockings, and NSAIDs and discontinuation of OCs. The lesions resolved after 6 weeks leaving bruiselike patches (erythema contusiformis).

EN is a delayed-type hypersensitivity reaction, causing inflammation on the fat (panniculitis) most commonly on the shins, but it can also occur on the arms, face, neck, and thighs. It is the most common type of panniculitis and is usually seen more often in women from the second to fourth decade of life. Erythematous tender nodules in crops commonly located on the shins are the characteristic physical finding. Systemic symptoms can occur including fever, malaise, and joint pain. The lesions usually last up to 6-8 weeks and may leave bruiselike patches or postinflammatory hyperpigmentation that can take months to improve.1

The diagnosis of EN usually is made by physical examination and natural history. In unusual severe cases or lesions in atypical locations, a skin biopsy is indicated. Histologic examination of one of the lesions reveals a septal panniculitis without vasculitis. Miescher’s radial granulomas (grouped macrophages around neutrophils or septa-like spaces) often are present and are a characteristic feature of EN.

EN can be triggered by different types of infections such as streptococcus, mycoplasma, tuberculosis, or bacterial gastroenteritis; medications such as OCs, sulfonamides, iodides, penicillin, or bromides; medical conditions that include inflammatory bowel disease, pregnancy, or sarcoidosis; or neutrophilic dermatosis and malignancy such as leukemia and Hodgkin disease.2,3 A third of the cases are idiopathic. In children, streptococcal infections are responsible for most cases of EN.4

Recommended work-up to investigate possible triggers includes a CBC with differential, sedimentation rate, CRP, ASO titers or anti-DNase B titers, tuberculin skin test or interferon-gamma TB test and a chest X ray. If there are any other symptoms, physical signs, or risk factors are present for the other not so common causes, further ancillary testing may be warranted.

Erythematous nodules and papules on the shin in children are commonly caused by arthropod bites also known as papular urticaria. These lesions are pruritic rather than tender and usually respond to topical corticosteroids and oral antihistamines. Subcutaneous bacterial, fungal, or atypical mycobacterial infections can present with tender nodules that can ulcerate and drain on the shins, feet, or any other body part. These patients may have a history of immunodeficiency and usually systemic symptoms of infection are present. Cutaneous polyarteritis nodosa (PAN) also can present with tender nodules on the legs but these lesions usually necrose and ulcerate and may be associated with livedo racemosa, a transient or persistent, blotchy, reddish-blue to purple, netlike cyanotic pattern. On pathology, PAN presents with necrotizing medium vessel vasculitis. Malignant nodules also can occur on the shin. Pathology will show atypical cells. Other forms of panniculitis, such as erythema induratum and pancreatic panniculitis, can present with tender nodules but these lesions usually occur on the calves and ulcerate.

Dr. Catalina Matiz

Management of EN starts with treating the underlying infection or stopping the causative medication. Initial measures include bed rest, leg elevation, compression bandages, and NSAIDs. Potassium iodide is a very effective therapy as it may control the symptoms within 24 hours. When there is no response to the above, or the patient has severe symptoms, a short course of systemic glucocorticoids can be started. Other medications for recalcitrant or recurrent lesions include colchicine, dapsone, or hydroxychloroquine.
 

 

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

1. Panniculitis, in “Dermatology,” 3rd ed. (Philadelphia: Elsevier Saunders, 2012, p. 1641).

2. Arthritis Rheum. 2000 Mar;43(3):584-92.

3. J Clin Oncol. 2007 Sep 1;25(25):4011-2.

4. Turk J Pediatr. 2014 Mar-Apr;56(2):144-9.

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Laboratory work revealed a normal CBC and differential, an elevated C-reactive protein (CRP) and sedimentation rate (ESR), negative antistreptolysin O (ASO) titers, negative pregnancy test, a normal urinalysis, and negative blood, throat, and urine cultures. A chest x-ray also was negative as well as angiotensin-converting enzyme (ACE) levels. Tuberculosis interferon-gamma release essay was negative.

The patient was diagnosed with erythema nodosum (EN), based on physical exam and history of the lesions. In her particular case, infectious causes including streptococcus infection, tuberculosis, and coccidioidomycosis were ruled out. There were no x-ray findings that suggested sarcoidosis and her ACE level was within normal limits. The pregnancy test also was negative. Given her recent start on OCs, this was thought to be the cause of the lesions.

She was treated with elevation, compression stockings, and NSAIDs and discontinuation of OCs. The lesions resolved after 6 weeks leaving bruiselike patches (erythema contusiformis).

EN is a delayed-type hypersensitivity reaction, causing inflammation on the fat (panniculitis) most commonly on the shins, but it can also occur on the arms, face, neck, and thighs. It is the most common type of panniculitis and is usually seen more often in women from the second to fourth decade of life. Erythematous tender nodules in crops commonly located on the shins are the characteristic physical finding. Systemic symptoms can occur including fever, malaise, and joint pain. The lesions usually last up to 6-8 weeks and may leave bruiselike patches or postinflammatory hyperpigmentation that can take months to improve.1

The diagnosis of EN usually is made by physical examination and natural history. In unusual severe cases or lesions in atypical locations, a skin biopsy is indicated. Histologic examination of one of the lesions reveals a septal panniculitis without vasculitis. Miescher’s radial granulomas (grouped macrophages around neutrophils or septa-like spaces) often are present and are a characteristic feature of EN.

EN can be triggered by different types of infections such as streptococcus, mycoplasma, tuberculosis, or bacterial gastroenteritis; medications such as OCs, sulfonamides, iodides, penicillin, or bromides; medical conditions that include inflammatory bowel disease, pregnancy, or sarcoidosis; or neutrophilic dermatosis and malignancy such as leukemia and Hodgkin disease.2,3 A third of the cases are idiopathic. In children, streptococcal infections are responsible for most cases of EN.4

Recommended work-up to investigate possible triggers includes a CBC with differential, sedimentation rate, CRP, ASO titers or anti-DNase B titers, tuberculin skin test or interferon-gamma TB test and a chest X ray. If there are any other symptoms, physical signs, or risk factors are present for the other not so common causes, further ancillary testing may be warranted.

Erythematous nodules and papules on the shin in children are commonly caused by arthropod bites also known as papular urticaria. These lesions are pruritic rather than tender and usually respond to topical corticosteroids and oral antihistamines. Subcutaneous bacterial, fungal, or atypical mycobacterial infections can present with tender nodules that can ulcerate and drain on the shins, feet, or any other body part. These patients may have a history of immunodeficiency and usually systemic symptoms of infection are present. Cutaneous polyarteritis nodosa (PAN) also can present with tender nodules on the legs but these lesions usually necrose and ulcerate and may be associated with livedo racemosa, a transient or persistent, blotchy, reddish-blue to purple, netlike cyanotic pattern. On pathology, PAN presents with necrotizing medium vessel vasculitis. Malignant nodules also can occur on the shin. Pathology will show atypical cells. Other forms of panniculitis, such as erythema induratum and pancreatic panniculitis, can present with tender nodules but these lesions usually occur on the calves and ulcerate.

Dr. Catalina Matiz

Management of EN starts with treating the underlying infection or stopping the causative medication. Initial measures include bed rest, leg elevation, compression bandages, and NSAIDs. Potassium iodide is a very effective therapy as it may control the symptoms within 24 hours. When there is no response to the above, or the patient has severe symptoms, a short course of systemic glucocorticoids can be started. Other medications for recalcitrant or recurrent lesions include colchicine, dapsone, or hydroxychloroquine.
 

 

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

1. Panniculitis, in “Dermatology,” 3rd ed. (Philadelphia: Elsevier Saunders, 2012, p. 1641).

2. Arthritis Rheum. 2000 Mar;43(3):584-92.

3. J Clin Oncol. 2007 Sep 1;25(25):4011-2.

4. Turk J Pediatr. 2014 Mar-Apr;56(2):144-9.

 

Laboratory work revealed a normal CBC and differential, an elevated C-reactive protein (CRP) and sedimentation rate (ESR), negative antistreptolysin O (ASO) titers, negative pregnancy test, a normal urinalysis, and negative blood, throat, and urine cultures. A chest x-ray also was negative as well as angiotensin-converting enzyme (ACE) levels. Tuberculosis interferon-gamma release essay was negative.

The patient was diagnosed with erythema nodosum (EN), based on physical exam and history of the lesions. In her particular case, infectious causes including streptococcus infection, tuberculosis, and coccidioidomycosis were ruled out. There were no x-ray findings that suggested sarcoidosis and her ACE level was within normal limits. The pregnancy test also was negative. Given her recent start on OCs, this was thought to be the cause of the lesions.

She was treated with elevation, compression stockings, and NSAIDs and discontinuation of OCs. The lesions resolved after 6 weeks leaving bruiselike patches (erythema contusiformis).

EN is a delayed-type hypersensitivity reaction, causing inflammation on the fat (panniculitis) most commonly on the shins, but it can also occur on the arms, face, neck, and thighs. It is the most common type of panniculitis and is usually seen more often in women from the second to fourth decade of life. Erythematous tender nodules in crops commonly located on the shins are the characteristic physical finding. Systemic symptoms can occur including fever, malaise, and joint pain. The lesions usually last up to 6-8 weeks and may leave bruiselike patches or postinflammatory hyperpigmentation that can take months to improve.1

The diagnosis of EN usually is made by physical examination and natural history. In unusual severe cases or lesions in atypical locations, a skin biopsy is indicated. Histologic examination of one of the lesions reveals a septal panniculitis without vasculitis. Miescher’s radial granulomas (grouped macrophages around neutrophils or septa-like spaces) often are present and are a characteristic feature of EN.

EN can be triggered by different types of infections such as streptococcus, mycoplasma, tuberculosis, or bacterial gastroenteritis; medications such as OCs, sulfonamides, iodides, penicillin, or bromides; medical conditions that include inflammatory bowel disease, pregnancy, or sarcoidosis; or neutrophilic dermatosis and malignancy such as leukemia and Hodgkin disease.2,3 A third of the cases are idiopathic. In children, streptococcal infections are responsible for most cases of EN.4

Recommended work-up to investigate possible triggers includes a CBC with differential, sedimentation rate, CRP, ASO titers or anti-DNase B titers, tuberculin skin test or interferon-gamma TB test and a chest X ray. If there are any other symptoms, physical signs, or risk factors are present for the other not so common causes, further ancillary testing may be warranted.

Erythematous nodules and papules on the shin in children are commonly caused by arthropod bites also known as papular urticaria. These lesions are pruritic rather than tender and usually respond to topical corticosteroids and oral antihistamines. Subcutaneous bacterial, fungal, or atypical mycobacterial infections can present with tender nodules that can ulcerate and drain on the shins, feet, or any other body part. These patients may have a history of immunodeficiency and usually systemic symptoms of infection are present. Cutaneous polyarteritis nodosa (PAN) also can present with tender nodules on the legs but these lesions usually necrose and ulcerate and may be associated with livedo racemosa, a transient or persistent, blotchy, reddish-blue to purple, netlike cyanotic pattern. On pathology, PAN presents with necrotizing medium vessel vasculitis. Malignant nodules also can occur on the shin. Pathology will show atypical cells. Other forms of panniculitis, such as erythema induratum and pancreatic panniculitis, can present with tender nodules but these lesions usually occur on the calves and ulcerate.

Dr. Catalina Matiz

Management of EN starts with treating the underlying infection or stopping the causative medication. Initial measures include bed rest, leg elevation, compression bandages, and NSAIDs. Potassium iodide is a very effective therapy as it may control the symptoms within 24 hours. When there is no response to the above, or the patient has severe symptoms, a short course of systemic glucocorticoids can be started. Other medications for recalcitrant or recurrent lesions include colchicine, dapsone, or hydroxychloroquine.
 

 

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego.

References

1. Panniculitis, in “Dermatology,” 3rd ed. (Philadelphia: Elsevier Saunders, 2012, p. 1641).

2. Arthritis Rheum. 2000 Mar;43(3):584-92.

3. J Clin Oncol. 2007 Sep 1;25(25):4011-2.

4. Turk J Pediatr. 2014 Mar-Apr;56(2):144-9.

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A 16-year-old female came to the dermatology clinic for acne follow-up. She reported some improvement on her acne since she started taking OCs. She also had been using benzoyl peroxide and tretinoin on her face. In addition to the acne, she also wanted us to check some tender bumps she had been getting on her shins after she came back from a camping trip. Initially she thought they were bug bites, but the lesions were getting larger, more tender, and not improving with diphenhydramine. 

On further questioning, she reported not having had fevers, chills, weight loss, upper respiratory symptoms, sore throat, cough, or respiratory symptoms. There was no family history of any other conditions except for acne on her father's side. She reported being sexually active, being on birth control for about 2 months, and she denied missing any doses. Her last menstrual period was 1 week prior to the appointment.

The physical exam did not reveal acute distress. She was afebrile. On skin examination, she had comedones, papules and scars on her face, chest, and back. On her shins there were several erythematous tender nodules and plaques. There was no edema on her legs and pulses were present.

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Make The Diagnosis - August 2018

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Pityriasis lichenoides chronica (PLC) and pityriasis lichenoides et varioliformis acuta (PLEVA) are the chronic and acute forms, respectively, of pityriasis lichenoides, an uncommon clonal T-cell disorder. The cause is unknown, although associations with infections have been reported. Pityriasis lichenoides more commonly affects children or young adults, usually before age 30 years. The disease can occur in all races.

In PLEVA, erythematous to brown papules and macules, in various stages of evolution, appear suddenly and in crops. The trunk and flexural areas are most often affected, but lesions may become widespread and may be pruritic or painful. Lesions may crust, ulcerate, or become necrotic and can heal with scarring. In general, patients don’t have constitutional symptoms. Lesions tend to resolve spontaneously over 1-3 years.

Rarely, PLEVA may develop into a more severe form called febrile ulceronecrotic Mucha-Habermann disease, a dermatologic emergency. Patients (more commonly, young males) may present with high fever, malaise, and lymphadenopathy. Lesions become very painful, ulcerated, and necrotic, and extensive necrosis may be present. Changes in mental status, breathing difficulties, anemia, arthritis, abdominal pain, and sepsis may occur. Patients require hospitalization. There is a 25% mortality rate.

PLC is at the other end of this disease spectrum, representing the chronic, more mild stage of the disorder. Lesions present as indolent, asymptomatic, scaly macules and erythematous papules, favoring the trunk and proximal extremities. Lesions tend to be fewer in number than seen in PLEVA. They resolve over several months and may result in hypopigmentation, but usually don’t cause scarring. Patients may have long periods of remission between outbreaks. T-cell gene rearrangement may demonstrate monoclonality. PLC is generally considered a benign disease, although there are patients who have developed cutaneous T-cell lymphoma. For this reason, patients should be followed carefully for signs of malignant transformation.

Both forms share a common histologic picture. In PLEVA, focal parakeratosis and crusting is present. A dense, wedge-shaped infiltrate can be seen with prominent lymphocytic exocystosis in the epidermis. Necrotic keratinocytes are often seen. There may be spongiosis and intraepidermal vesicles. Extravasation of erythrocytes often occurs in the epidermis. PLC is histologically similar but far more subtle. There is less crusting, less spongiosis, fewer vesicles, and fewer necrotic keratinocytes. Generally, atypia of lymphocytes is absent.

Mucha-Habermann requires treatment with systemic steroids. Methotrexate, cyclosporine, or dapsone may be used as steroid-sparing agents. Upon treatment, lesions may resolve or revert back to more typical lesions of PLEVA. Treatment for PLEVA and PLC includes oral tetracycline or erythromycin, antihistamines (if pruritus is present), topical steroids, topical tacrolimus or pimecrolimus, or phototherapy. Low-dose weekly methotrexate may be helpful.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].


 

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Pityriasis lichenoides chronica (PLC) and pityriasis lichenoides et varioliformis acuta (PLEVA) are the chronic and acute forms, respectively, of pityriasis lichenoides, an uncommon clonal T-cell disorder. The cause is unknown, although associations with infections have been reported. Pityriasis lichenoides more commonly affects children or young adults, usually before age 30 years. The disease can occur in all races.

In PLEVA, erythematous to brown papules and macules, in various stages of evolution, appear suddenly and in crops. The trunk and flexural areas are most often affected, but lesions may become widespread and may be pruritic or painful. Lesions may crust, ulcerate, or become necrotic and can heal with scarring. In general, patients don’t have constitutional symptoms. Lesions tend to resolve spontaneously over 1-3 years.

Rarely, PLEVA may develop into a more severe form called febrile ulceronecrotic Mucha-Habermann disease, a dermatologic emergency. Patients (more commonly, young males) may present with high fever, malaise, and lymphadenopathy. Lesions become very painful, ulcerated, and necrotic, and extensive necrosis may be present. Changes in mental status, breathing difficulties, anemia, arthritis, abdominal pain, and sepsis may occur. Patients require hospitalization. There is a 25% mortality rate.

PLC is at the other end of this disease spectrum, representing the chronic, more mild stage of the disorder. Lesions present as indolent, asymptomatic, scaly macules and erythematous papules, favoring the trunk and proximal extremities. Lesions tend to be fewer in number than seen in PLEVA. They resolve over several months and may result in hypopigmentation, but usually don’t cause scarring. Patients may have long periods of remission between outbreaks. T-cell gene rearrangement may demonstrate monoclonality. PLC is generally considered a benign disease, although there are patients who have developed cutaneous T-cell lymphoma. For this reason, patients should be followed carefully for signs of malignant transformation.

Both forms share a common histologic picture. In PLEVA, focal parakeratosis and crusting is present. A dense, wedge-shaped infiltrate can be seen with prominent lymphocytic exocystosis in the epidermis. Necrotic keratinocytes are often seen. There may be spongiosis and intraepidermal vesicles. Extravasation of erythrocytes often occurs in the epidermis. PLC is histologically similar but far more subtle. There is less crusting, less spongiosis, fewer vesicles, and fewer necrotic keratinocytes. Generally, atypia of lymphocytes is absent.

Mucha-Habermann requires treatment with systemic steroids. Methotrexate, cyclosporine, or dapsone may be used as steroid-sparing agents. Upon treatment, lesions may resolve or revert back to more typical lesions of PLEVA. Treatment for PLEVA and PLC includes oral tetracycline or erythromycin, antihistamines (if pruritus is present), topical steroids, topical tacrolimus or pimecrolimus, or phototherapy. Low-dose weekly methotrexate may be helpful.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].


 

Pityriasis lichenoides chronica (PLC) and pityriasis lichenoides et varioliformis acuta (PLEVA) are the chronic and acute forms, respectively, of pityriasis lichenoides, an uncommon clonal T-cell disorder. The cause is unknown, although associations with infections have been reported. Pityriasis lichenoides more commonly affects children or young adults, usually before age 30 years. The disease can occur in all races.

In PLEVA, erythematous to brown papules and macules, in various stages of evolution, appear suddenly and in crops. The trunk and flexural areas are most often affected, but lesions may become widespread and may be pruritic or painful. Lesions may crust, ulcerate, or become necrotic and can heal with scarring. In general, patients don’t have constitutional symptoms. Lesions tend to resolve spontaneously over 1-3 years.

Rarely, PLEVA may develop into a more severe form called febrile ulceronecrotic Mucha-Habermann disease, a dermatologic emergency. Patients (more commonly, young males) may present with high fever, malaise, and lymphadenopathy. Lesions become very painful, ulcerated, and necrotic, and extensive necrosis may be present. Changes in mental status, breathing difficulties, anemia, arthritis, abdominal pain, and sepsis may occur. Patients require hospitalization. There is a 25% mortality rate.

PLC is at the other end of this disease spectrum, representing the chronic, more mild stage of the disorder. Lesions present as indolent, asymptomatic, scaly macules and erythematous papules, favoring the trunk and proximal extremities. Lesions tend to be fewer in number than seen in PLEVA. They resolve over several months and may result in hypopigmentation, but usually don’t cause scarring. Patients may have long periods of remission between outbreaks. T-cell gene rearrangement may demonstrate monoclonality. PLC is generally considered a benign disease, although there are patients who have developed cutaneous T-cell lymphoma. For this reason, patients should be followed carefully for signs of malignant transformation.

Both forms share a common histologic picture. In PLEVA, focal parakeratosis and crusting is present. A dense, wedge-shaped infiltrate can be seen with prominent lymphocytic exocystosis in the epidermis. Necrotic keratinocytes are often seen. There may be spongiosis and intraepidermal vesicles. Extravasation of erythrocytes often occurs in the epidermis. PLC is histologically similar but far more subtle. There is less crusting, less spongiosis, fewer vesicles, and fewer necrotic keratinocytes. Generally, atypia of lymphocytes is absent.

Mucha-Habermann requires treatment with systemic steroids. Methotrexate, cyclosporine, or dapsone may be used as steroid-sparing agents. Upon treatment, lesions may resolve or revert back to more typical lesions of PLEVA. Treatment for PLEVA and PLC includes oral tetracycline or erythromycin, antihistamines (if pruritus is present), topical steroids, topical tacrolimus or pimecrolimus, or phototherapy. Low-dose weekly methotrexate may be helpful.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].


 

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A 28-year-old white female with no significant past medical history presents with a 10-year history of asymptomatic erythematous papules and scaly patches that come and go. She has used topical steroids in the past.

Pityriasis lichenoides chronica (PLC).

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Pediatric Dermatology Consult - July 2018

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Streptococcal intertrigo is an inflammatory, superficial eruption of intertriginous skin caused by group A beta-hemolytic streptococci. Frequently misdiagnosed, streptococcal intertrigo more commonly affects infants and toddlers but is rarely reported, especially compared with other Streptococcus pyogenes infections, including impetigo, erysipelas, and cellulitis.1

Intertrigo, meaning “between” (inter) and “to rub” (terere) in Latin, describes any skin disorder involving two opposing skin surfaces that touch or rub to cause friction.2 The continuous chaffing, coupled with moisture trapped within the skin folds, leads to irritation and maceration, which provides an ideal environment for pathogens to thrive. Thus, frictional dermatitides that arise may become secondarily infected with one or more microorganisms, such as Candida albicans, Staphylococcus aureus, Streptococcus pyogenes, and even organisms less commonly associated with cutaneous infection, such as Proteus mirabilis.3

Courtesy Dr. Lawrence F. Eichenfield
Intertrigo caused by Streptococcus pyogenes is classically characterized by fiery erythema and moist maceration. Homogeneous in appearance, lesions are discretely demarcated and notably lack satellite lesions, unlike in candidal intertrigo. Distinctly foul odor and worsening or persistence of rash despite antifungal treatment also are helpful features in distinguishing streptococcal intertrigo from candidal intertrigo.4 These characteristics are essential to consider because streptococcal intertrigo is most commonly initially misdiagnosed as candidal intertrigo, which is the most common type of intertrigo.5

Streptococcal intertrigo may affect any intertriginous area, but most commonly it affects the folds of the neck; this is likely because of the combination of the deep folds that develop in shorter, infantile necks and the moisture from drool and saliva that pools in the area.5,6 In addition to these cervical folds, other intertriginous areas commonly are affected, including the inguinal, axillary, popliteal, posterior auricular, perianal, and genital folds.

Perianal streptococcal disease may present in a similar manner as streptococcal intertrigo, manifesting as well-demarcated, beefy red plaques in the skin folds around the anus and, in females, frequently perivaginally.7 Unlike streptococcal intertrigo, perianal streptococcal disease is often characterized by pain, pruritus, and fissuring of the involved area.8 It is associated with pharyngeal colonization of group A beta-hemolytic streptococci.7

Allison Han
Patients may be well appearing, or they may present with irritability and low-grade fever; only one case in the literature has described development of bacteremia secondary to cervical streptococcal intertrigo that had developed in a 12-month-old infant.1

Diagnosis is straight forward and may be confirmed by a positive streptococcal rapid antigen test of swab specimens of one or more surfaces of affected skin or by culture from a skin swab yielding growth of the organism.1,5 Skin biopsy is not necessary. If the index of suspicion for candida is high, a potassium hydroxide preparation and culture may be performed. Checking serum anti-DNase B antibodies, antistreptolysin O, and pharyngeal cultures is often unrevealing.9 A urinalysis may be performed to assess for poststreptococcal glomerulonephritis if the patient later develops facial or orbital edema, hypertension, hematuria, or lethargy.9

Treatment consists of systemic antistreptococcal therapy; oral amoxicillin and penicillin frequently have been used.9 Moisture in the area should be reduced with application of absorptive powders and physical barriers, such as zinc oxide, after gentle cleansing of the area.5

Dr. Lawrence F. Eichenfield
Of note, in patients with underlying atopic dermatitis, secondary infection with Streptococcus pyogenes may exacerbate atopic dermatitis, resulting in a flare of inflammation that persists even after appropriate therapy of the Streptococcus pyogenes. In these patients, it is reasonable and beneficial to treat with concomitant topical corticosteroids.9Streptococcus pyogenes infection also has been identified as a trigger for guttate psoriasis. Thus, if coin-shaped, erythematous lesions with scale are appreciated, a diagnosis of guttate psoriasis should be considered and the presence of a preceding streptococcal infection inquired about.5 Although islands of erythema and scale may resemble satellite lesions, it is important to include guttate psoriasis in the differential and not confound these entities because they lead to favoring of differing diagnoses and treatment options.4

Other diagnoses to consider when evaluating dermatitides affecting skin folds include: other infectious causes, which may be ruled out by fungal or bacterial culture; inverse psoriasis, which will frequently demonstrate scale; atopic dermatitis, which will be pruritic with history of atopy; irritant or contact dermatitis, which will often have correlating clinical history; seborrheic dermatitis, which will often involve greasiness and scale; and less commonly, acrodermatitis enteropathica, which will be accompanied by diarrhea and hair loss.2,9 Scabies also may be on the differential if the patient endorses severe pruritus with close contacts with similar symptoms.
 

Ms. Han is a medical student at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and a professor of dermatology and pediatrics at the university. They had no conflicts of interest or disclosures to report.


References

1. Pediatr Dermatol. 2014 Mar-Apr;31(2):e71-2.

2. Clin Dermatol. 2011 Mar-Apr;29(2):173-9.

3. Pediatrics. 2003 Dec;112(6 pt 1):1427-9.

4. BMJ Case Rep. 2018 Mar 20. doi: 10.1136/bcr-2018-224179.

5. Pediatr Infect Dis J. 2012 Aug;31(8):872-3.

6. J Pediatr. 2015 May;166(5):1318.

7. J Pediatr. 2015 Sep;167(3):687-93.e1-2.

8. Pediatrics in Review. 1991;12(8):248-55.

9. J Pediatr. 2017 May;184:230-1.e1.

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Streptococcal intertrigo is an inflammatory, superficial eruption of intertriginous skin caused by group A beta-hemolytic streptococci. Frequently misdiagnosed, streptococcal intertrigo more commonly affects infants and toddlers but is rarely reported, especially compared with other Streptococcus pyogenes infections, including impetigo, erysipelas, and cellulitis.1

Intertrigo, meaning “between” (inter) and “to rub” (terere) in Latin, describes any skin disorder involving two opposing skin surfaces that touch or rub to cause friction.2 The continuous chaffing, coupled with moisture trapped within the skin folds, leads to irritation and maceration, which provides an ideal environment for pathogens to thrive. Thus, frictional dermatitides that arise may become secondarily infected with one or more microorganisms, such as Candida albicans, Staphylococcus aureus, Streptococcus pyogenes, and even organisms less commonly associated with cutaneous infection, such as Proteus mirabilis.3

Courtesy Dr. Lawrence F. Eichenfield
Intertrigo caused by Streptococcus pyogenes is classically characterized by fiery erythema and moist maceration. Homogeneous in appearance, lesions are discretely demarcated and notably lack satellite lesions, unlike in candidal intertrigo. Distinctly foul odor and worsening or persistence of rash despite antifungal treatment also are helpful features in distinguishing streptococcal intertrigo from candidal intertrigo.4 These characteristics are essential to consider because streptococcal intertrigo is most commonly initially misdiagnosed as candidal intertrigo, which is the most common type of intertrigo.5

Streptococcal intertrigo may affect any intertriginous area, but most commonly it affects the folds of the neck; this is likely because of the combination of the deep folds that develop in shorter, infantile necks and the moisture from drool and saliva that pools in the area.5,6 In addition to these cervical folds, other intertriginous areas commonly are affected, including the inguinal, axillary, popliteal, posterior auricular, perianal, and genital folds.

Perianal streptococcal disease may present in a similar manner as streptococcal intertrigo, manifesting as well-demarcated, beefy red plaques in the skin folds around the anus and, in females, frequently perivaginally.7 Unlike streptococcal intertrigo, perianal streptococcal disease is often characterized by pain, pruritus, and fissuring of the involved area.8 It is associated with pharyngeal colonization of group A beta-hemolytic streptococci.7

Allison Han
Patients may be well appearing, or they may present with irritability and low-grade fever; only one case in the literature has described development of bacteremia secondary to cervical streptococcal intertrigo that had developed in a 12-month-old infant.1

Diagnosis is straight forward and may be confirmed by a positive streptococcal rapid antigen test of swab specimens of one or more surfaces of affected skin or by culture from a skin swab yielding growth of the organism.1,5 Skin biopsy is not necessary. If the index of suspicion for candida is high, a potassium hydroxide preparation and culture may be performed. Checking serum anti-DNase B antibodies, antistreptolysin O, and pharyngeal cultures is often unrevealing.9 A urinalysis may be performed to assess for poststreptococcal glomerulonephritis if the patient later develops facial or orbital edema, hypertension, hematuria, or lethargy.9

Treatment consists of systemic antistreptococcal therapy; oral amoxicillin and penicillin frequently have been used.9 Moisture in the area should be reduced with application of absorptive powders and physical barriers, such as zinc oxide, after gentle cleansing of the area.5

Dr. Lawrence F. Eichenfield
Of note, in patients with underlying atopic dermatitis, secondary infection with Streptococcus pyogenes may exacerbate atopic dermatitis, resulting in a flare of inflammation that persists even after appropriate therapy of the Streptococcus pyogenes. In these patients, it is reasonable and beneficial to treat with concomitant topical corticosteroids.9Streptococcus pyogenes infection also has been identified as a trigger for guttate psoriasis. Thus, if coin-shaped, erythematous lesions with scale are appreciated, a diagnosis of guttate psoriasis should be considered and the presence of a preceding streptococcal infection inquired about.5 Although islands of erythema and scale may resemble satellite lesions, it is important to include guttate psoriasis in the differential and not confound these entities because they lead to favoring of differing diagnoses and treatment options.4

Other diagnoses to consider when evaluating dermatitides affecting skin folds include: other infectious causes, which may be ruled out by fungal or bacterial culture; inverse psoriasis, which will frequently demonstrate scale; atopic dermatitis, which will be pruritic with history of atopy; irritant or contact dermatitis, which will often have correlating clinical history; seborrheic dermatitis, which will often involve greasiness and scale; and less commonly, acrodermatitis enteropathica, which will be accompanied by diarrhea and hair loss.2,9 Scabies also may be on the differential if the patient endorses severe pruritus with close contacts with similar symptoms.
 

Ms. Han is a medical student at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and a professor of dermatology and pediatrics at the university. They had no conflicts of interest or disclosures to report.


References

1. Pediatr Dermatol. 2014 Mar-Apr;31(2):e71-2.

2. Clin Dermatol. 2011 Mar-Apr;29(2):173-9.

3. Pediatrics. 2003 Dec;112(6 pt 1):1427-9.

4. BMJ Case Rep. 2018 Mar 20. doi: 10.1136/bcr-2018-224179.

5. Pediatr Infect Dis J. 2012 Aug;31(8):872-3.

6. J Pediatr. 2015 May;166(5):1318.

7. J Pediatr. 2015 Sep;167(3):687-93.e1-2.

8. Pediatrics in Review. 1991;12(8):248-55.

9. J Pediatr. 2017 May;184:230-1.e1.

 

Streptococcal intertrigo is an inflammatory, superficial eruption of intertriginous skin caused by group A beta-hemolytic streptococci. Frequently misdiagnosed, streptococcal intertrigo more commonly affects infants and toddlers but is rarely reported, especially compared with other Streptococcus pyogenes infections, including impetigo, erysipelas, and cellulitis.1

Intertrigo, meaning “between” (inter) and “to rub” (terere) in Latin, describes any skin disorder involving two opposing skin surfaces that touch or rub to cause friction.2 The continuous chaffing, coupled with moisture trapped within the skin folds, leads to irritation and maceration, which provides an ideal environment for pathogens to thrive. Thus, frictional dermatitides that arise may become secondarily infected with one or more microorganisms, such as Candida albicans, Staphylococcus aureus, Streptococcus pyogenes, and even organisms less commonly associated with cutaneous infection, such as Proteus mirabilis.3

Courtesy Dr. Lawrence F. Eichenfield
Intertrigo caused by Streptococcus pyogenes is classically characterized by fiery erythema and moist maceration. Homogeneous in appearance, lesions are discretely demarcated and notably lack satellite lesions, unlike in candidal intertrigo. Distinctly foul odor and worsening or persistence of rash despite antifungal treatment also are helpful features in distinguishing streptococcal intertrigo from candidal intertrigo.4 These characteristics are essential to consider because streptococcal intertrigo is most commonly initially misdiagnosed as candidal intertrigo, which is the most common type of intertrigo.5

Streptococcal intertrigo may affect any intertriginous area, but most commonly it affects the folds of the neck; this is likely because of the combination of the deep folds that develop in shorter, infantile necks and the moisture from drool and saliva that pools in the area.5,6 In addition to these cervical folds, other intertriginous areas commonly are affected, including the inguinal, axillary, popliteal, posterior auricular, perianal, and genital folds.

Perianal streptococcal disease may present in a similar manner as streptococcal intertrigo, manifesting as well-demarcated, beefy red plaques in the skin folds around the anus and, in females, frequently perivaginally.7 Unlike streptococcal intertrigo, perianal streptococcal disease is often characterized by pain, pruritus, and fissuring of the involved area.8 It is associated with pharyngeal colonization of group A beta-hemolytic streptococci.7

Allison Han
Patients may be well appearing, or they may present with irritability and low-grade fever; only one case in the literature has described development of bacteremia secondary to cervical streptococcal intertrigo that had developed in a 12-month-old infant.1

Diagnosis is straight forward and may be confirmed by a positive streptococcal rapid antigen test of swab specimens of one or more surfaces of affected skin or by culture from a skin swab yielding growth of the organism.1,5 Skin biopsy is not necessary. If the index of suspicion for candida is high, a potassium hydroxide preparation and culture may be performed. Checking serum anti-DNase B antibodies, antistreptolysin O, and pharyngeal cultures is often unrevealing.9 A urinalysis may be performed to assess for poststreptococcal glomerulonephritis if the patient later develops facial or orbital edema, hypertension, hematuria, or lethargy.9

Treatment consists of systemic antistreptococcal therapy; oral amoxicillin and penicillin frequently have been used.9 Moisture in the area should be reduced with application of absorptive powders and physical barriers, such as zinc oxide, after gentle cleansing of the area.5

Dr. Lawrence F. Eichenfield
Of note, in patients with underlying atopic dermatitis, secondary infection with Streptococcus pyogenes may exacerbate atopic dermatitis, resulting in a flare of inflammation that persists even after appropriate therapy of the Streptococcus pyogenes. In these patients, it is reasonable and beneficial to treat with concomitant topical corticosteroids.9Streptococcus pyogenes infection also has been identified as a trigger for guttate psoriasis. Thus, if coin-shaped, erythematous lesions with scale are appreciated, a diagnosis of guttate psoriasis should be considered and the presence of a preceding streptococcal infection inquired about.5 Although islands of erythema and scale may resemble satellite lesions, it is important to include guttate psoriasis in the differential and not confound these entities because they lead to favoring of differing diagnoses and treatment options.4

Other diagnoses to consider when evaluating dermatitides affecting skin folds include: other infectious causes, which may be ruled out by fungal or bacterial culture; inverse psoriasis, which will frequently demonstrate scale; atopic dermatitis, which will be pruritic with history of atopy; irritant or contact dermatitis, which will often have correlating clinical history; seborrheic dermatitis, which will often involve greasiness and scale; and less commonly, acrodermatitis enteropathica, which will be accompanied by diarrhea and hair loss.2,9 Scabies also may be on the differential if the patient endorses severe pruritus with close contacts with similar symptoms.
 

Ms. Han is a medical student at the University of California, San Diego. Dr. Eichenfield is chief of pediatric and adolescent dermatology at Rady Children’s Hospital–San Diego. He is vice chair of the department of dermatology and a professor of dermatology and pediatrics at the university. They had no conflicts of interest or disclosures to report.


References

1. Pediatr Dermatol. 2014 Mar-Apr;31(2):e71-2.

2. Clin Dermatol. 2011 Mar-Apr;29(2):173-9.

3. Pediatrics. 2003 Dec;112(6 pt 1):1427-9.

4. BMJ Case Rep. 2018 Mar 20. doi: 10.1136/bcr-2018-224179.

5. Pediatr Infect Dis J. 2012 Aug;31(8):872-3.

6. J Pediatr. 2015 May;166(5):1318.

7. J Pediatr. 2015 Sep;167(3):687-93.e1-2.

8. Pediatrics in Review. 1991;12(8):248-55.

9. J Pediatr. 2017 May;184:230-1.e1.

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An 8-week-old male with a history of cradle cap presented for a second evaluation of an erythematous rash on the neck that started 1.5 weeks before, and it had since worsened. The parents note that their infant has been more irritable, but they otherwise deny any fever, diarrhea, constipation, or decrease in oral intake.

Courtesy Dr. Lawrence F. Eichenfield

The patient’s first evaluation had been 3 days prior; nystatin cream was prescribed, and the parents applied it twice a day but without improvement to the rash. The patient also had a rash behind the ears bilaterally, which was treated with hydrocortisone 2.5% ointment with some improvement

On physical exam, the central neck is covered by a bright, beefy red, erythematous plaque with distinct borders and strong odor. There is faint scale and superficial desquamation between the skin folds. There are no surrounding papules or pustules. The patient’s chin is moist with drool. In the postauricular skin folds bilaterally, there are fainter but still erythematous plaques with mild scale.

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Make the Diagnosis - June 2018

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Steatocystoma multiplex is an uncommon inherited condition in which multiple lesions are formed, most commonly appearing on the trunk, axillae, and groin. Different types of steatocystoma multiplex have been described: localized, generalized, facial, acral, and suppurative (in which the lesions resemble hidradenitis suppurativa).

This condition is autosomal dominant and is linked to defects in KRT17 gene, which instructs the production of keratin 17. However, some cases of steatocystoma multiplex occur sporadically with no mutation in the KRT17 gene; in them, the cause is unknown. Steatocystoma multiplex may be associated with eruptive vellus hair cysts and pachyonychia congenita (nail and teeth abnormalities and palmoplantar keratoderma). Lesions often appear during adolescence, when an individual hits puberty. Hormones likely influence the development of the cysts from the pilosebaceous unit. If there is a single steatocystoma, it is called steatocystoma simplex.

Steatocystomas do not resolve on their own. The small, benign cysts are located fairly superficial in the dermis. If punctured, they drain a yellow, oily liquid sebum. Lesions may become inflamed and may heal with scarring, as in acne. They may be treated by incision and drainage or excision to remove the cyst wall. Electrosurgery and cryotherapy may be used. Oral antibiotics may improve inflamed lesions. There are reports in the literature in which isotretinoin has helped; however, it is not curative. In some cases, the lesions can reoccur and may even be worse.
 

Case and photo submitted by: Donna Bilu Martin, MD; Premier Dermatology, MD; Aventura, Fla.


Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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Steatocystoma multiplex is an uncommon inherited condition in which multiple lesions are formed, most commonly appearing on the trunk, axillae, and groin. Different types of steatocystoma multiplex have been described: localized, generalized, facial, acral, and suppurative (in which the lesions resemble hidradenitis suppurativa).

This condition is autosomal dominant and is linked to defects in KRT17 gene, which instructs the production of keratin 17. However, some cases of steatocystoma multiplex occur sporadically with no mutation in the KRT17 gene; in them, the cause is unknown. Steatocystoma multiplex may be associated with eruptive vellus hair cysts and pachyonychia congenita (nail and teeth abnormalities and palmoplantar keratoderma). Lesions often appear during adolescence, when an individual hits puberty. Hormones likely influence the development of the cysts from the pilosebaceous unit. If there is a single steatocystoma, it is called steatocystoma simplex.

Steatocystomas do not resolve on their own. The small, benign cysts are located fairly superficial in the dermis. If punctured, they drain a yellow, oily liquid sebum. Lesions may become inflamed and may heal with scarring, as in acne. They may be treated by incision and drainage or excision to remove the cyst wall. Electrosurgery and cryotherapy may be used. Oral antibiotics may improve inflamed lesions. There are reports in the literature in which isotretinoin has helped; however, it is not curative. In some cases, the lesions can reoccur and may even be worse.
 

Case and photo submitted by: Donna Bilu Martin, MD; Premier Dermatology, MD; Aventura, Fla.


Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

 

Steatocystoma multiplex is an uncommon inherited condition in which multiple lesions are formed, most commonly appearing on the trunk, axillae, and groin. Different types of steatocystoma multiplex have been described: localized, generalized, facial, acral, and suppurative (in which the lesions resemble hidradenitis suppurativa).

This condition is autosomal dominant and is linked to defects in KRT17 gene, which instructs the production of keratin 17. However, some cases of steatocystoma multiplex occur sporadically with no mutation in the KRT17 gene; in them, the cause is unknown. Steatocystoma multiplex may be associated with eruptive vellus hair cysts and pachyonychia congenita (nail and teeth abnormalities and palmoplantar keratoderma). Lesions often appear during adolescence, when an individual hits puberty. Hormones likely influence the development of the cysts from the pilosebaceous unit. If there is a single steatocystoma, it is called steatocystoma simplex.

Steatocystomas do not resolve on their own. The small, benign cysts are located fairly superficial in the dermis. If punctured, they drain a yellow, oily liquid sebum. Lesions may become inflamed and may heal with scarring, as in acne. They may be treated by incision and drainage or excision to remove the cyst wall. Electrosurgery and cryotherapy may be used. Oral antibiotics may improve inflamed lesions. There are reports in the literature in which isotretinoin has helped; however, it is not curative. In some cases, the lesions can reoccur and may even be worse.
 

Case and photo submitted by: Donna Bilu Martin, MD; Premier Dermatology, MD; Aventura, Fla.


Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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A 38-year-old female with no significant past medical history presented with a greater than 20-year history of numerous cystic nodules on her trunk, buttocks, and upper extremities. She is unsure of her immediate family's medical history, but states her niece has similar lesions. She received no previous treatment for the lesions.

 
 
 

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Make the Diagnosis - May 2018

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Pediculosis pubis, also known as pubic lice, or “crabs,” is an infestation of Phthirus pubis. Crab lice are spread sexually and through close skin contact, as well as contaminated clothes and bedding. Adult lice can live up to 36 hours away from its host. Pubic areas most commonly are affected, although other hair-bearing parts of the body often are affected, including eyelashes.

Courtesy Dr. Maria Hicks and Dr. Donna Bilu Martin

Pruritus can be severe. Secondary bacterial infections may occur as maculae ceruleae, or blue-colored macules, on the skin. The lice are visible to the naked eye and are approximately 1 mm in length. They have a crablike appearance, six legs, and a wide body. Nits may be present on the hair shaft. Unlike hair casts, which can be moved up and down along the hair shaft, nits firmly adhere to the hair. Diagnosis should prompt a workup for other sexually transmitted diseases, including HIV.

Treatment for patients and their sexual partners include permethrin topically; and laundering of clothing and bedding. Lice on the eyelashes can be treated with 8 days of twice-daily applications of petrolatum. Ivermectin can be used when topical therapy fails, although this is an off-label treatment (not approved by the Food and Drug Administration).

Pediculosis corporis – body lice or clothing lice – is also known as “vagabond’s disease” and is caused by Pediculus humanus var corporis. Body lice lay their eggs in clothing seams and can live in clothing for up to 1 month without feeding on human blood. Often homeless individuals and those living in overcrowded areas can be affected. The louse and nits also are visible to the naked eye. They have a longer, narrower body than Phthirus pubis and are more similar in appearance to head lice. They rarely are found on the skin.

Body lice may carry disease such as epidemic typhus, relapsing fever, and trench fever or endocarditis. Permethrin is the most widely used treatment to kill both lice and ova. Other treatments include Malathion, Lindane, and Crotamiton. Clothing and bedding should be laundered.

Scabies is a mite infestation caused by Sarcoptes scabiei. Unlike lice, scabies often affects the hands and feet. Characteristic linear burrows may be seen in the finger web spaces. The circle of Hebra describes the areas commonly infected by mites: axillae, antecubital fossa, wrists, hands, and the groin. Pruritus may be severe and worse at night. Patients may be afflicted with both lice and scabies at the same time. Mites are not visible to the naked eye but can be seen microscopically. Topical permethrin cream is used most often for treatment. All household contacts should be treated at the same time. As in louse infestations, clothing and bedding should be laundered. Ivermectin can be used for crusted scabies, although this is an off-label treatment.

This case and photo were submitted by Maria Hicks, MD, Advanced Dermatology and Cosmetic Surgery, Tampa, and Dr. Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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Pediculosis pubis, also known as pubic lice, or “crabs,” is an infestation of Phthirus pubis. Crab lice are spread sexually and through close skin contact, as well as contaminated clothes and bedding. Adult lice can live up to 36 hours away from its host. Pubic areas most commonly are affected, although other hair-bearing parts of the body often are affected, including eyelashes.

Courtesy Dr. Maria Hicks and Dr. Donna Bilu Martin

Pruritus can be severe. Secondary bacterial infections may occur as maculae ceruleae, or blue-colored macules, on the skin. The lice are visible to the naked eye and are approximately 1 mm in length. They have a crablike appearance, six legs, and a wide body. Nits may be present on the hair shaft. Unlike hair casts, which can be moved up and down along the hair shaft, nits firmly adhere to the hair. Diagnosis should prompt a workup for other sexually transmitted diseases, including HIV.

Treatment for patients and their sexual partners include permethrin topically; and laundering of clothing and bedding. Lice on the eyelashes can be treated with 8 days of twice-daily applications of petrolatum. Ivermectin can be used when topical therapy fails, although this is an off-label treatment (not approved by the Food and Drug Administration).

Pediculosis corporis – body lice or clothing lice – is also known as “vagabond’s disease” and is caused by Pediculus humanus var corporis. Body lice lay their eggs in clothing seams and can live in clothing for up to 1 month without feeding on human blood. Often homeless individuals and those living in overcrowded areas can be affected. The louse and nits also are visible to the naked eye. They have a longer, narrower body than Phthirus pubis and are more similar in appearance to head lice. They rarely are found on the skin.

Body lice may carry disease such as epidemic typhus, relapsing fever, and trench fever or endocarditis. Permethrin is the most widely used treatment to kill both lice and ova. Other treatments include Malathion, Lindane, and Crotamiton. Clothing and bedding should be laundered.

Scabies is a mite infestation caused by Sarcoptes scabiei. Unlike lice, scabies often affects the hands and feet. Characteristic linear burrows may be seen in the finger web spaces. The circle of Hebra describes the areas commonly infected by mites: axillae, antecubital fossa, wrists, hands, and the groin. Pruritus may be severe and worse at night. Patients may be afflicted with both lice and scabies at the same time. Mites are not visible to the naked eye but can be seen microscopically. Topical permethrin cream is used most often for treatment. All household contacts should be treated at the same time. As in louse infestations, clothing and bedding should be laundered. Ivermectin can be used for crusted scabies, although this is an off-label treatment.

This case and photo were submitted by Maria Hicks, MD, Advanced Dermatology and Cosmetic Surgery, Tampa, and Dr. Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

Pediculosis pubis, also known as pubic lice, or “crabs,” is an infestation of Phthirus pubis. Crab lice are spread sexually and through close skin contact, as well as contaminated clothes and bedding. Adult lice can live up to 36 hours away from its host. Pubic areas most commonly are affected, although other hair-bearing parts of the body often are affected, including eyelashes.

Courtesy Dr. Maria Hicks and Dr. Donna Bilu Martin

Pruritus can be severe. Secondary bacterial infections may occur as maculae ceruleae, or blue-colored macules, on the skin. The lice are visible to the naked eye and are approximately 1 mm in length. They have a crablike appearance, six legs, and a wide body. Nits may be present on the hair shaft. Unlike hair casts, which can be moved up and down along the hair shaft, nits firmly adhere to the hair. Diagnosis should prompt a workup for other sexually transmitted diseases, including HIV.

Treatment for patients and their sexual partners include permethrin topically; and laundering of clothing and bedding. Lice on the eyelashes can be treated with 8 days of twice-daily applications of petrolatum. Ivermectin can be used when topical therapy fails, although this is an off-label treatment (not approved by the Food and Drug Administration).

Pediculosis corporis – body lice or clothing lice – is also known as “vagabond’s disease” and is caused by Pediculus humanus var corporis. Body lice lay their eggs in clothing seams and can live in clothing for up to 1 month without feeding on human blood. Often homeless individuals and those living in overcrowded areas can be affected. The louse and nits also are visible to the naked eye. They have a longer, narrower body than Phthirus pubis and are more similar in appearance to head lice. They rarely are found on the skin.

Body lice may carry disease such as epidemic typhus, relapsing fever, and trench fever or endocarditis. Permethrin is the most widely used treatment to kill both lice and ova. Other treatments include Malathion, Lindane, and Crotamiton. Clothing and bedding should be laundered.

Scabies is a mite infestation caused by Sarcoptes scabiei. Unlike lice, scabies often affects the hands and feet. Characteristic linear burrows may be seen in the finger web spaces. The circle of Hebra describes the areas commonly infected by mites: axillae, antecubital fossa, wrists, hands, and the groin. Pruritus may be severe and worse at night. Patients may be afflicted with both lice and scabies at the same time. Mites are not visible to the naked eye but can be seen microscopically. Topical permethrin cream is used most often for treatment. All household contacts should be treated at the same time. As in louse infestations, clothing and bedding should be laundered. Ivermectin can be used for crusted scabies, although this is an off-label treatment.

This case and photo were submitted by Maria Hicks, MD, Advanced Dermatology and Cosmetic Surgery, Tampa, and Dr. Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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A 40-year-old HIV-positive male presented with a 1-month history of severely pruritic papules on his chest. The patient reported that he "removes bugs" from his skin. Microscopic examination of a hair clipping was performed.  
Make the Diagnosis:

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Make the Diagnosis - May 2018

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Erythema infectiosum, also known as fifth disease, is a viral exanthem linked to parvovirus B19. Generally, school-aged children are most often affected. Infections are more likely in late winter and early spring. The virus is spread via respiratory secretions, blood products, and transmission from mother to fetus. The cutaneous findings occur about 10 days after exposure to the virus. By that time, the risk of being contagious is low.

Courtesy Dr. Donna Bilu-Martin
Classically, asymptomatic erythema first appears on the cheeks, resembling a “slapped cheek” appearance. Lacy, reticulated erythema then appears on the trunk and extremities 1-4 days later. Lesions often fade over the course of 2-3 weeks, but may persist for longer periods of time. Lesions may be recurrent after exposure to heat, bathing, sunlight, or upon physical activity. Younger children may exhibit mild fever, headache, runny nose, and arthralgias prior to the cutaneous eruption. Arthralgias are more common in adolescents and adults, who may even develop severe arthritis.

Healthy individuals have no sequelae from fifth disease and require no treatment. However, in patients with hemoglobinopathies, such as sickle cell disease, an aplastic crisis can be triggered. In patients with deficient immune systems, parvovirus B19 may cause infection and anemia, requiring hospitalization. Pregnant women exposed to parvovirus B19 are at risk for hydrops fetalis and rarely, fetal malformations or fetal demise. Other uncommon associations include hepatitis, vasculitides, and neurologic disease.

Dr. Donna Bilu Martin
Exanthem subitum, or roseola, classically presents in children aged 6 months–3 years. Three to five days of high fevers is followed by pink papules over the entire body. Papular acrodermatitis presents as erythematous papules on the extremities, buttocks, and face, classically sparing the trunk. It is associated with enterovirus, respiratory viruses, and Epstein-Barr virus. Livedo reticularis occurs more often in healthy young women and presents as a persistent, lacy erythema.
 

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]. This case and photo were submitted by Dr. Bilu Martin.

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Erythema infectiosum, also known as fifth disease, is a viral exanthem linked to parvovirus B19. Generally, school-aged children are most often affected. Infections are more likely in late winter and early spring. The virus is spread via respiratory secretions, blood products, and transmission from mother to fetus. The cutaneous findings occur about 10 days after exposure to the virus. By that time, the risk of being contagious is low.

Courtesy Dr. Donna Bilu-Martin
Classically, asymptomatic erythema first appears on the cheeks, resembling a “slapped cheek” appearance. Lacy, reticulated erythema then appears on the trunk and extremities 1-4 days later. Lesions often fade over the course of 2-3 weeks, but may persist for longer periods of time. Lesions may be recurrent after exposure to heat, bathing, sunlight, or upon physical activity. Younger children may exhibit mild fever, headache, runny nose, and arthralgias prior to the cutaneous eruption. Arthralgias are more common in adolescents and adults, who may even develop severe arthritis.

Healthy individuals have no sequelae from fifth disease and require no treatment. However, in patients with hemoglobinopathies, such as sickle cell disease, an aplastic crisis can be triggered. In patients with deficient immune systems, parvovirus B19 may cause infection and anemia, requiring hospitalization. Pregnant women exposed to parvovirus B19 are at risk for hydrops fetalis and rarely, fetal malformations or fetal demise. Other uncommon associations include hepatitis, vasculitides, and neurologic disease.

Dr. Donna Bilu Martin
Exanthem subitum, or roseola, classically presents in children aged 6 months–3 years. Three to five days of high fevers is followed by pink papules over the entire body. Papular acrodermatitis presents as erythematous papules on the extremities, buttocks, and face, classically sparing the trunk. It is associated with enterovirus, respiratory viruses, and Epstein-Barr virus. Livedo reticularis occurs more often in healthy young women and presents as a persistent, lacy erythema.
 

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]. This case and photo were submitted by Dr. Bilu Martin.

 

Erythema infectiosum, also known as fifth disease, is a viral exanthem linked to parvovirus B19. Generally, school-aged children are most often affected. Infections are more likely in late winter and early spring. The virus is spread via respiratory secretions, blood products, and transmission from mother to fetus. The cutaneous findings occur about 10 days after exposure to the virus. By that time, the risk of being contagious is low.

Courtesy Dr. Donna Bilu-Martin
Classically, asymptomatic erythema first appears on the cheeks, resembling a “slapped cheek” appearance. Lacy, reticulated erythema then appears on the trunk and extremities 1-4 days later. Lesions often fade over the course of 2-3 weeks, but may persist for longer periods of time. Lesions may be recurrent after exposure to heat, bathing, sunlight, or upon physical activity. Younger children may exhibit mild fever, headache, runny nose, and arthralgias prior to the cutaneous eruption. Arthralgias are more common in adolescents and adults, who may even develop severe arthritis.

Healthy individuals have no sequelae from fifth disease and require no treatment. However, in patients with hemoglobinopathies, such as sickle cell disease, an aplastic crisis can be triggered. In patients with deficient immune systems, parvovirus B19 may cause infection and anemia, requiring hospitalization. Pregnant women exposed to parvovirus B19 are at risk for hydrops fetalis and rarely, fetal malformations or fetal demise. Other uncommon associations include hepatitis, vasculitides, and neurologic disease.

Dr. Donna Bilu Martin
Exanthem subitum, or roseola, classically presents in children aged 6 months–3 years. Three to five days of high fevers is followed by pink papules over the entire body. Papular acrodermatitis presents as erythematous papules on the extremities, buttocks, and face, classically sparing the trunk. It is associated with enterovirus, respiratory viruses, and Epstein-Barr virus. Livedo reticularis occurs more often in healthy young women and presents as a persistent, lacy erythema.
 

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]. This case and photo were submitted by Dr. Bilu Martin.

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Courtesy Dr. Donna Bilu-Martin
A 4-year-old healthy male presented with a lacy, reticular eruption on the abdomen and legs for 2 days. He had a mild fever and red cheeks a few days prior to presentation. The patient attends preschool.

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Make The Diagnosis - April 2018

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Herpes zoster, also known as shingles, is caused by a reactivation of varicella-zoster virus. Once an individual has been exposed to varicella-zoster virus, either from primary infection (chickenpox) or vaccination, the virus remains dormant in dorsal root ganglion cells. It may become reactivated at a later time, which results in herpes zoster. Typically, immunosuppression (hematologic malignancy and HIV infection) and age are factors that play a role in reactivation, although young people may develop shingles as well. Older age increases the incidence of herpes zoster.

Courtesy Dr. Donna Bilu Martin
Classically, herpes zoster occurs unilaterally within the distribution of a nerve and affects the dermatome of skin associated with the nerve. The trunk is most commonly involved.

More than 90% of patients will experience a prodrome of pain, burning, or tingling in the dermatome prior to the development of cutaneous lesions. Occasionally, there will be no symptoms prior. Papules and plaques begin to form, which quickly develop into vesicles and blisters. After a few days, lesions become crusted. Bullae or necrosis may occur in more severe cases. Typically, the condition resolves in 2-3 weeks, but can take 6 weeks or longer in elderly patients. In zoster sine herpete, patients have pain but no skin lesions.

In typical herpes zoster, lesions can be scattered outside the dermatome as well. When more than 20 lesions are scattered outside the area of primary or adjacent dermatomes, this is defined as disseminated herpes zoster. This occurs more commonly in debilitated or immune-compromised individuals. The outlying vesicles are often singular, not grouped, and resemble the “dew drop on a rose petal” look of varicella-zoster lesions. Dissemination necessitates systemic antiviral therapy, preferably intravenous followed by oral treatment once stable. Central nervous system and pulmonary involvement can occur.

Complications of zoster can occur. Postherpetic neuralgia and pain is more common in patients over the age of 50 and may become chronic. Ramsay Hunt syndrome may result in facial paralysis and hearing loss when there is involvement of the facial or auditory nerve. Occasionally, inflammatory lesions can occur within the affected area after the infection has resolved. Secondary bacterial infection, scarring, and motor paralysis can occur.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]. This case and photo were submitted by Dr. Bilu Martin.

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Herpes zoster, also known as shingles, is caused by a reactivation of varicella-zoster virus. Once an individual has been exposed to varicella-zoster virus, either from primary infection (chickenpox) or vaccination, the virus remains dormant in dorsal root ganglion cells. It may become reactivated at a later time, which results in herpes zoster. Typically, immunosuppression (hematologic malignancy and HIV infection) and age are factors that play a role in reactivation, although young people may develop shingles as well. Older age increases the incidence of herpes zoster.

Courtesy Dr. Donna Bilu Martin
Classically, herpes zoster occurs unilaterally within the distribution of a nerve and affects the dermatome of skin associated with the nerve. The trunk is most commonly involved.

More than 90% of patients will experience a prodrome of pain, burning, or tingling in the dermatome prior to the development of cutaneous lesions. Occasionally, there will be no symptoms prior. Papules and plaques begin to form, which quickly develop into vesicles and blisters. After a few days, lesions become crusted. Bullae or necrosis may occur in more severe cases. Typically, the condition resolves in 2-3 weeks, but can take 6 weeks or longer in elderly patients. In zoster sine herpete, patients have pain but no skin lesions.

In typical herpes zoster, lesions can be scattered outside the dermatome as well. When more than 20 lesions are scattered outside the area of primary or adjacent dermatomes, this is defined as disseminated herpes zoster. This occurs more commonly in debilitated or immune-compromised individuals. The outlying vesicles are often singular, not grouped, and resemble the “dew drop on a rose petal” look of varicella-zoster lesions. Dissemination necessitates systemic antiviral therapy, preferably intravenous followed by oral treatment once stable. Central nervous system and pulmonary involvement can occur.

Complications of zoster can occur. Postherpetic neuralgia and pain is more common in patients over the age of 50 and may become chronic. Ramsay Hunt syndrome may result in facial paralysis and hearing loss when there is involvement of the facial or auditory nerve. Occasionally, inflammatory lesions can occur within the affected area after the infection has resolved. Secondary bacterial infection, scarring, and motor paralysis can occur.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]. This case and photo were submitted by Dr. Bilu Martin.

 

Herpes zoster, also known as shingles, is caused by a reactivation of varicella-zoster virus. Once an individual has been exposed to varicella-zoster virus, either from primary infection (chickenpox) or vaccination, the virus remains dormant in dorsal root ganglion cells. It may become reactivated at a later time, which results in herpes zoster. Typically, immunosuppression (hematologic malignancy and HIV infection) and age are factors that play a role in reactivation, although young people may develop shingles as well. Older age increases the incidence of herpes zoster.

Courtesy Dr. Donna Bilu Martin
Classically, herpes zoster occurs unilaterally within the distribution of a nerve and affects the dermatome of skin associated with the nerve. The trunk is most commonly involved.

More than 90% of patients will experience a prodrome of pain, burning, or tingling in the dermatome prior to the development of cutaneous lesions. Occasionally, there will be no symptoms prior. Papules and plaques begin to form, which quickly develop into vesicles and blisters. After a few days, lesions become crusted. Bullae or necrosis may occur in more severe cases. Typically, the condition resolves in 2-3 weeks, but can take 6 weeks or longer in elderly patients. In zoster sine herpete, patients have pain but no skin lesions.

In typical herpes zoster, lesions can be scattered outside the dermatome as well. When more than 20 lesions are scattered outside the area of primary or adjacent dermatomes, this is defined as disseminated herpes zoster. This occurs more commonly in debilitated or immune-compromised individuals. The outlying vesicles are often singular, not grouped, and resemble the “dew drop on a rose petal” look of varicella-zoster lesions. Dissemination necessitates systemic antiviral therapy, preferably intravenous followed by oral treatment once stable. Central nervous system and pulmonary involvement can occur.

Complications of zoster can occur. Postherpetic neuralgia and pain is more common in patients over the age of 50 and may become chronic. Ramsay Hunt syndrome may result in facial paralysis and hearing loss when there is involvement of the facial or auditory nerve. Occasionally, inflammatory lesions can occur within the affected area after the infection has resolved. Secondary bacterial infection, scarring, and motor paralysis can occur.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]. This case and photo were submitted by Dr. Bilu Martin.

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A healthy 70-year-old white male presented with an 8-day history of fatigue and a tingling, erythematous plaque with crusting on the left flank. Four days after the flank lesions appeared, he developed vesicles with an erythematous base on the right abdomen and back. There were more than 20 vesicles present on the abdomen and back, but there were no lesions on other parts of the body.

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Make the Diagnosis - March 2018

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Familial benign chronic pemphigus, also known as Hailey-Hailey disease, is an uncommon autosomal dominant genetic condition. A mutation in the calcium ATPase (ATP2C1) gene on chromosome 3q21 interferes with calcium signaling and results in a loss of keratinocyte adhesion. Generally, the onset of the condition is in the second or third decade. There are two clinical subtypes of the disease: segmental type 1 and segmental type 2.

Courtesy of Dr. Donna Bilu Martin
Lesions present as flaccid bullae, vesicles, or erosions that commonly affect the neck, axillae, and flexures. Often there is maceration and fissuring and bullae are no longer intact. Crusting and secondary bacterial, viral, or fungal infection may occur. Lesions can heal with postinflammatory pigmentation but usually do not leave scarring. Heat and sweating may exacerbate the lesions, and they become malodorous. Squamous cell carcinoma has been reported in lesions of Hailey-Hailey, so regular surveillance is important.

Histology reveals groups of acantholytic cells that resemble a “dilapidated brick wall.” Direct immunofluorescence is negative, unlike pemphigus vulgaris.

Dr. Donna Bilu Martin
Treatment may be challenging. Topical and systemic corticosteroids often improve lesions. Topical calcineurin inhibitors can be used as steroid sparing agents. Antimicrobial cleansers can be helpful. If secondary infection is present, topical and systemic antibiotics (with Staphylococcus aureus as the most common bacteria involved), antivirals, and antifungals may be added. For extensive disease, cyclosporine and oral retinoids have been used but are not consistent in their efficacy. CO2 laser and surgical grafting can be used for unresponsive disease.

As hyperhidrosis is a known aggravating factor, injection with botulinum toxin (this is off-label use not yet approved by the Food and Drug Administration) in affected areas to decrease sweating has been reported to be effective.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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Familial benign chronic pemphigus, also known as Hailey-Hailey disease, is an uncommon autosomal dominant genetic condition. A mutation in the calcium ATPase (ATP2C1) gene on chromosome 3q21 interferes with calcium signaling and results in a loss of keratinocyte adhesion. Generally, the onset of the condition is in the second or third decade. There are two clinical subtypes of the disease: segmental type 1 and segmental type 2.

Courtesy of Dr. Donna Bilu Martin
Lesions present as flaccid bullae, vesicles, or erosions that commonly affect the neck, axillae, and flexures. Often there is maceration and fissuring and bullae are no longer intact. Crusting and secondary bacterial, viral, or fungal infection may occur. Lesions can heal with postinflammatory pigmentation but usually do not leave scarring. Heat and sweating may exacerbate the lesions, and they become malodorous. Squamous cell carcinoma has been reported in lesions of Hailey-Hailey, so regular surveillance is important.

Histology reveals groups of acantholytic cells that resemble a “dilapidated brick wall.” Direct immunofluorescence is negative, unlike pemphigus vulgaris.

Dr. Donna Bilu Martin
Treatment may be challenging. Topical and systemic corticosteroids often improve lesions. Topical calcineurin inhibitors can be used as steroid sparing agents. Antimicrobial cleansers can be helpful. If secondary infection is present, topical and systemic antibiotics (with Staphylococcus aureus as the most common bacteria involved), antivirals, and antifungals may be added. For extensive disease, cyclosporine and oral retinoids have been used but are not consistent in their efficacy. CO2 laser and surgical grafting can be used for unresponsive disease.

As hyperhidrosis is a known aggravating factor, injection with botulinum toxin (this is off-label use not yet approved by the Food and Drug Administration) in affected areas to decrease sweating has been reported to be effective.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

 

Familial benign chronic pemphigus, also known as Hailey-Hailey disease, is an uncommon autosomal dominant genetic condition. A mutation in the calcium ATPase (ATP2C1) gene on chromosome 3q21 interferes with calcium signaling and results in a loss of keratinocyte adhesion. Generally, the onset of the condition is in the second or third decade. There are two clinical subtypes of the disease: segmental type 1 and segmental type 2.

Courtesy of Dr. Donna Bilu Martin
Lesions present as flaccid bullae, vesicles, or erosions that commonly affect the neck, axillae, and flexures. Often there is maceration and fissuring and bullae are no longer intact. Crusting and secondary bacterial, viral, or fungal infection may occur. Lesions can heal with postinflammatory pigmentation but usually do not leave scarring. Heat and sweating may exacerbate the lesions, and they become malodorous. Squamous cell carcinoma has been reported in lesions of Hailey-Hailey, so regular surveillance is important.

Histology reveals groups of acantholytic cells that resemble a “dilapidated brick wall.” Direct immunofluorescence is negative, unlike pemphigus vulgaris.

Dr. Donna Bilu Martin
Treatment may be challenging. Topical and systemic corticosteroids often improve lesions. Topical calcineurin inhibitors can be used as steroid sparing agents. Antimicrobial cleansers can be helpful. If secondary infection is present, topical and systemic antibiotics (with Staphylococcus aureus as the most common bacteria involved), antivirals, and antifungals may be added. For extensive disease, cyclosporine and oral retinoids have been used but are not consistent in their efficacy. CO2 laser and surgical grafting can be used for unresponsive disease.

As hyperhidrosis is a known aggravating factor, injection with botulinum toxin (this is off-label use not yet approved by the Food and Drug Administration) in affected areas to decrease sweating has been reported to be effective.

This case and photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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A 39-year-old healthy black woman presented with itchy, painful lesions in the bilateral axillae and groin. The lesions have come and gone for 15 years and flare when the patient perspires. Her mother and grandmother have the same condition.

Courtesy of Dr. Donna Bilu Martin
Upon physical examination, hyperpigmented plaques with minimal crusting were present in the axillae and intertriginous plaques with crusting were present in the groin. The lesions have responded to triamcinolone in the past. Biopsies for hematoxylin and eosin staining and direct immunofluorescence were performed.

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Make the Diagnosis - February 2018

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Neurofibromatosis (NF) is an autosomal dominant genetic neurocutaneous disorder. There are eight subtypes of NF: NF type 1-7 and NF-NOS, or not otherwise specified. Neurofibromatosis type 1 (NF-1), or von Recklinghausen disease, is the most common and is a result of a genetic mutation on chromosome 17 that is involved in producing a protein called neurofibromin. Neurofibromin is a tumor suppressor that suppresses products of ras proto-oncogenes. When it is absent, tumor progression may occur. 

Courtesy Dr. Parteek Singla and Dr. Donna Bilu Martin

Von Recklinghausen NF-1 appears in childhood, usually by age 10. Diagnosis requires the presence of at least 2 of the following 7 criteria:
•Six or more café au lait macules measuring 5 mm in diameter or greater in prepubertal children and measuring greater than 15 mm in postpubertal children.
•Axillary or inguinal freckling (Crowe’s sign).
•Two or more neurofibromas or one plexiform neurofibroma.
•Optic nerve glioma.
•Two or more iris hamartomas (Lisch nodules).
•Sphenoid dysplasia or long-bone abnormalities, such as pseudoarthrosis.
•First degree relative with NF-1.

The diagnosis is usually made via physical examination. Supportive tests include an ophthalmologic exam to detect Lisch nodules and cataracts. A neurological evaluation is essential. Imaging examinations can identify bony abnormalities and tumor growths. Also, genetic testing to identify genetic mutations can be performed.

Dr. Donna Bilu Martin
Patients may develop tumors (malignant peripheral nerve sheath tumors, pheochromocytomas, central nervous system tumors), seizures, learning difficulties, scoliosis, and juvenile chronic myelogenous leukemia. Hypertension may result from renal artery stenosis or pheochromocytomas. As a result, individuals with NF-1 require regular follow up to assess for plexiform neurofibromas, evaluate blood pressure, growth, skeletal changes, learning development, and eye exams. Age appropriate cancer screening is highly recommended. 

Neurofibromatosis type 2 results from a genetic mutation located on chromosome 22 that produces a protein called merlin and occurs in adolescence. Acoustic or vestibular neuromas may occur; these interfere with the transmission of sound and maintaining balance. Symptoms include gradual hearing loss, tinnitus, poor balance, and headaches. Radiosurgery and cochlear implants have shown a role for symptomatic treatment in patients with NF-2. 

This case and photo were submitted by Parteek Singla, MD, of the division of dermatology at Washington University and Barnes Jewish Hospital, both in St. Louis, and by Dr. Bilu Martin.


Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]
 

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Neurofibromatosis (NF) is an autosomal dominant genetic neurocutaneous disorder. There are eight subtypes of NF: NF type 1-7 and NF-NOS, or not otherwise specified. Neurofibromatosis type 1 (NF-1), or von Recklinghausen disease, is the most common and is a result of a genetic mutation on chromosome 17 that is involved in producing a protein called neurofibromin. Neurofibromin is a tumor suppressor that suppresses products of ras proto-oncogenes. When it is absent, tumor progression may occur. 

Courtesy Dr. Parteek Singla and Dr. Donna Bilu Martin

Von Recklinghausen NF-1 appears in childhood, usually by age 10. Diagnosis requires the presence of at least 2 of the following 7 criteria:
•Six or more café au lait macules measuring 5 mm in diameter or greater in prepubertal children and measuring greater than 15 mm in postpubertal children.
•Axillary or inguinal freckling (Crowe’s sign).
•Two or more neurofibromas or one plexiform neurofibroma.
•Optic nerve glioma.
•Two or more iris hamartomas (Lisch nodules).
•Sphenoid dysplasia or long-bone abnormalities, such as pseudoarthrosis.
•First degree relative with NF-1.

The diagnosis is usually made via physical examination. Supportive tests include an ophthalmologic exam to detect Lisch nodules and cataracts. A neurological evaluation is essential. Imaging examinations can identify bony abnormalities and tumor growths. Also, genetic testing to identify genetic mutations can be performed.

Dr. Donna Bilu Martin
Patients may develop tumors (malignant peripheral nerve sheath tumors, pheochromocytomas, central nervous system tumors), seizures, learning difficulties, scoliosis, and juvenile chronic myelogenous leukemia. Hypertension may result from renal artery stenosis or pheochromocytomas. As a result, individuals with NF-1 require regular follow up to assess for plexiform neurofibromas, evaluate blood pressure, growth, skeletal changes, learning development, and eye exams. Age appropriate cancer screening is highly recommended. 

Neurofibromatosis type 2 results from a genetic mutation located on chromosome 22 that produces a protein called merlin and occurs in adolescence. Acoustic or vestibular neuromas may occur; these interfere with the transmission of sound and maintaining balance. Symptoms include gradual hearing loss, tinnitus, poor balance, and headaches. Radiosurgery and cochlear implants have shown a role for symptomatic treatment in patients with NF-2. 

This case and photo were submitted by Parteek Singla, MD, of the division of dermatology at Washington University and Barnes Jewish Hospital, both in St. Louis, and by Dr. Bilu Martin.


Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]
 

Neurofibromatosis (NF) is an autosomal dominant genetic neurocutaneous disorder. There are eight subtypes of NF: NF type 1-7 and NF-NOS, or not otherwise specified. Neurofibromatosis type 1 (NF-1), or von Recklinghausen disease, is the most common and is a result of a genetic mutation on chromosome 17 that is involved in producing a protein called neurofibromin. Neurofibromin is a tumor suppressor that suppresses products of ras proto-oncogenes. When it is absent, tumor progression may occur. 

Courtesy Dr. Parteek Singla and Dr. Donna Bilu Martin

Von Recklinghausen NF-1 appears in childhood, usually by age 10. Diagnosis requires the presence of at least 2 of the following 7 criteria:
•Six or more café au lait macules measuring 5 mm in diameter or greater in prepubertal children and measuring greater than 15 mm in postpubertal children.
•Axillary or inguinal freckling (Crowe’s sign).
•Two or more neurofibromas or one plexiform neurofibroma.
•Optic nerve glioma.
•Two or more iris hamartomas (Lisch nodules).
•Sphenoid dysplasia or long-bone abnormalities, such as pseudoarthrosis.
•First degree relative with NF-1.

The diagnosis is usually made via physical examination. Supportive tests include an ophthalmologic exam to detect Lisch nodules and cataracts. A neurological evaluation is essential. Imaging examinations can identify bony abnormalities and tumor growths. Also, genetic testing to identify genetic mutations can be performed.

Patients may develop tumors (malignant peripheral nerve sheath tumors, pheochromocytomas, central nervous system tumors), seizures, learning difficulties, scoliosis, and juvenile chronic myelogenous leukemia. Hypertension may result from renal artery stenosis or pheochromocytomas. As a result, individuals with NF-1 require regular follow up to assess for plexiform neurofibromas, evaluate blood pressure, growth, skeletal changes, learning development, and eye exams. Age appropriate cancer screening is highly recommended. 

Neurofibromatosis type 2 results from a genetic mutation located on chromosome 22 that produces a protein called merlin and occurs in adolescence. Acoustic or vestibular neuromas may occur; these interfere with the transmission of sound and maintaining balance. Symptoms include gradual hearing loss, tinnitus, poor balance, and headaches. Radiosurgery and cochlear implants have shown a role for symptomatic treatment in patients with NF-2. 

This case and photo were submitted by Parteek Singla, MD, of the division of dermatology at Washington University and Barnes Jewish Hospital, both in St. Louis, and by Dr. Bilu Martin.


Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected]
 

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Courtesy Dr. Parteek Singla and Dr. Donna Bilu Martin
A 64-year-old female presented for a routine full body skin exam. On examination, multiple flesh colored papules were present on her trunk, arms, and legs. The lesions have been present since childhood. She also had multiple café au-lait macules and hyperpigmented macules in the axilla. Her mother had similar lesions on her skin.

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Make the diagnosis - January 2018

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Make the diagnosis - January 2018

Cutaneous lupus erythematosus can be classified into acute, subacute, and chronic lesions. Chronic cutaneous lupus, or discoid lupus erythematosus (DLE), may occur independently of or in combination with systemic lupus erythematosus (SLE). They are one of the more common skin presentations seen in lupus. Young adults are typically affected, with a female-to-male ratio of 2:1. Progression from DLE to SLE is uncommon. However, patients with SLE will frequently develop discoid lesions.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
Lesions generally occur on the head and neck, with scalp and ears (conchal bowls) frequently affected. DLE lesions often begin as erythematous papules or plaques that may become scaly and heal with atrophy, scarring and dyspigmentation (often central hypopigmentation with peripheral hyperpigmentation). Follicular plugging is often seen in lesions. Erosions may occur. A small percentage of patients may have mucosal involvement, including the lips. Sun exposure may have a role in the development of lesions, although lesions may also occur in non–sun exposed areas. Less commonly, DLE may be generalized and involve the trunk and extremities, in addition to the head and neck. Scarring alopecia can be present on the scalp. Scarring may become disfiguring.

The differential diagnosis includes: subacute cutaneous lupus, lichen planus, seborrheic dermatitis, Jessner’s lymphocytic infiltrate, polymorphous light eruption, rosacea, granuloma faciale, and sarcoidosis. Histology of DLE may reveal hyperkeratosis, a thin epidermis with effacement of the rete ridges, a lichenoid and vacuolar interface dermatitis, and follicular plugging. Damaged keratinocytes called colloid bodies may be present. Increased mucin and thickening of the basement membrane are commonly seen. Active lesions will exhibit more of an inflammatory infiltrate. Direct immunofluorescence of lesional skin is positive in more than 75% of cases.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
Treatment includes sunscreen and avoidance of sun exposure. Potent or superpotent topical corticosteroids, as well as lesional injections of triamcinolone are helpful. Although, generally, it is not advised to use a high-potency steroid on the face, it can be helpful in DLE. Application should be limited to affected areas for short periods of time, with frequent monitoring for possible side effects. Topical calcineurin inhibitors can be used in addition to topical corticosteroids. If systemic treatment is indicated, hydroxychloroquine is first line. Short-term oral corticosteroid treatment can be used while transitioning to other systemic medications. Our patient had negative serologies and responded to high-dose topical steroids with complete clearing of cutaneous lesions.
This case and the photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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Cutaneous lupus erythematosus can be classified into acute, subacute, and chronic lesions. Chronic cutaneous lupus, or discoid lupus erythematosus (DLE), may occur independently of or in combination with systemic lupus erythematosus (SLE). They are one of the more common skin presentations seen in lupus. Young adults are typically affected, with a female-to-male ratio of 2:1. Progression from DLE to SLE is uncommon. However, patients with SLE will frequently develop discoid lesions.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
Lesions generally occur on the head and neck, with scalp and ears (conchal bowls) frequently affected. DLE lesions often begin as erythematous papules or plaques that may become scaly and heal with atrophy, scarring and dyspigmentation (often central hypopigmentation with peripheral hyperpigmentation). Follicular plugging is often seen in lesions. Erosions may occur. A small percentage of patients may have mucosal involvement, including the lips. Sun exposure may have a role in the development of lesions, although lesions may also occur in non–sun exposed areas. Less commonly, DLE may be generalized and involve the trunk and extremities, in addition to the head and neck. Scarring alopecia can be present on the scalp. Scarring may become disfiguring.

The differential diagnosis includes: subacute cutaneous lupus, lichen planus, seborrheic dermatitis, Jessner’s lymphocytic infiltrate, polymorphous light eruption, rosacea, granuloma faciale, and sarcoidosis. Histology of DLE may reveal hyperkeratosis, a thin epidermis with effacement of the rete ridges, a lichenoid and vacuolar interface dermatitis, and follicular plugging. Damaged keratinocytes called colloid bodies may be present. Increased mucin and thickening of the basement membrane are commonly seen. Active lesions will exhibit more of an inflammatory infiltrate. Direct immunofluorescence of lesional skin is positive in more than 75% of cases.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
Treatment includes sunscreen and avoidance of sun exposure. Potent or superpotent topical corticosteroids, as well as lesional injections of triamcinolone are helpful. Although, generally, it is not advised to use a high-potency steroid on the face, it can be helpful in DLE. Application should be limited to affected areas for short periods of time, with frequent monitoring for possible side effects. Topical calcineurin inhibitors can be used in addition to topical corticosteroids. If systemic treatment is indicated, hydroxychloroquine is first line. Short-term oral corticosteroid treatment can be used while transitioning to other systemic medications. Our patient had negative serologies and responded to high-dose topical steroids with complete clearing of cutaneous lesions.
This case and the photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

Cutaneous lupus erythematosus can be classified into acute, subacute, and chronic lesions. Chronic cutaneous lupus, or discoid lupus erythematosus (DLE), may occur independently of or in combination with systemic lupus erythematosus (SLE). They are one of the more common skin presentations seen in lupus. Young adults are typically affected, with a female-to-male ratio of 2:1. Progression from DLE to SLE is uncommon. However, patients with SLE will frequently develop discoid lesions.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
Lesions generally occur on the head and neck, with scalp and ears (conchal bowls) frequently affected. DLE lesions often begin as erythematous papules or plaques that may become scaly and heal with atrophy, scarring and dyspigmentation (often central hypopigmentation with peripheral hyperpigmentation). Follicular plugging is often seen in lesions. Erosions may occur. A small percentage of patients may have mucosal involvement, including the lips. Sun exposure may have a role in the development of lesions, although lesions may also occur in non–sun exposed areas. Less commonly, DLE may be generalized and involve the trunk and extremities, in addition to the head and neck. Scarring alopecia can be present on the scalp. Scarring may become disfiguring.

The differential diagnosis includes: subacute cutaneous lupus, lichen planus, seborrheic dermatitis, Jessner’s lymphocytic infiltrate, polymorphous light eruption, rosacea, granuloma faciale, and sarcoidosis. Histology of DLE may reveal hyperkeratosis, a thin epidermis with effacement of the rete ridges, a lichenoid and vacuolar interface dermatitis, and follicular plugging. Damaged keratinocytes called colloid bodies may be present. Increased mucin and thickening of the basement membrane are commonly seen. Active lesions will exhibit more of an inflammatory infiltrate. Direct immunofluorescence of lesional skin is positive in more than 75% of cases.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
Treatment includes sunscreen and avoidance of sun exposure. Potent or superpotent topical corticosteroids, as well as lesional injections of triamcinolone are helpful. Although, generally, it is not advised to use a high-potency steroid on the face, it can be helpful in DLE. Application should be limited to affected areas for short periods of time, with frequent monitoring for possible side effects. Topical calcineurin inhibitors can be used in addition to topical corticosteroids. If systemic treatment is indicated, hydroxychloroquine is first line. Short-term oral corticosteroid treatment can be used while transitioning to other systemic medications. Our patient had negative serologies and responded to high-dose topical steroids with complete clearing of cutaneous lesions.
This case and the photo were submitted by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to [email protected].

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Make the diagnosis - January 2018
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A 32-year-old male with no significant past medical history presented with a 2-year history of asymptomatic perioral lesions. On physical examination, multiple erythematous to hypopigmented atrophic plaques with peripheral hyperpigmentation were present.

Courtesy Dr. Donna Bilu Martin, Premier Dermatology, MD, Aventura, Fla.
No lesions were present elsewhere, including in the scalp and concha of the ears. The patient denied systemic symptoms such as arthritis, photosensitivity, or oral ulcers.

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