Cushing syndrome from percutaneous absorption of 1% hydrocortisone ointment in Netherton syndrome.

Netherton syndrome is a congenital skin disease associated with decreased skin barrier function and increased percutaneous absorption. We report an 11-year-old boy with Netherton syndrome who developed Cushing syndrome after application of 1% hydrocortisone ointment to his entire body for more than 1 year. This presentation illustrates that even low-potency steroid ointments should be used with caution in Netherton syndrome and warns about the use of long-term topical medications with potential systemic side effects when used in large quantities in any chronic skin disease.

Infantile acne as the initial sign of an adrenocortical tumor.

A 23-month-old boy with persistent acne since 6 months of age had signs of virilization and accelerated growth. Hormone evaluation indicated increased levels of dehydroepiandrosterone (DHEA) and testosterone. Ultrasound and abdominal computed tomographic scan revealed a large adrenal mass consistent with an adrenocortical tumor. The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormones and no subsequent recurrence. Although rare, childhood adrenocortical tumors have a poor prognosis, with the majority of tumors having regional and metastatic disease. Because early diagnosis and complete surgical excision improve prognosis, children with refractory infantile acne should be evaluated for signs of virilization and accelerated growth. Laboratory evaluation should include luteinizing hormone, follicle-stimulating hormone, total and free testosterone cortisol, DHEA, and its sulfate (DHEA-S). Elevated levels of DHEA and DHEA-S should prompt an aggressive diagnostic evaluation for an adrenocortical tumor.

Dermatologic disorders associated with chronic hepatitis C: effect of interferon therapy.

Chronic hepatitis C virus infection (HCV) is associated with extrahepatic manifestations, including such dermatologic conditions as mixed cryoglobulinemia, porphyria cutanea tarda, and lichen planus. Patients with chronic HCV and extrahepatic manifestations are often excluded from clinical trials evaluating interferon (IFN) therapy due to concerns about poor response, adverse events, and toxicity. Thus, data regarding the efficacy of IFN not only on the underlying chronic HCV, but also on extrahepatic manifestations, are limited in these patients. Case reports suggest that the response of dermatologic extrahepatic manifestations to IFN in patients with chronic HCV is highly variable. This review summarizes available data on dermatologic conditions associated with chronic HCV and their response to IFN therapy.

Congenital plaque-type glomuvenous malformations presenting in childhood.

BACKGROUND: Glomuvenous malformations (GVMs) are now considered a separate entity from venous malformations. The rarest type of GVM is the generalized congenital plaque-type GVM. OBSERVATIONS: We present 10 new cases of congenital plaque-type GVM and describe their clinical progression and treatment. Mutations in the glomulin gene were found in those patients who participated in the genetic study. CONCLUSIONS: Congenital plaque-type GVMs are unique in their congenital nature, extensive distribution, difficult to diagnose and treat, and progressive involvement after birth. Most cases are familial, yet affected relatives usually have only minor lesions. The lesions of congenital plaque-type GVM are severe, visible at birth, and usually mistaken for extensive venous malformations. Vascular malformations are divided by hemodynamic type into slow-flow and fast-flow lesions. Slow-flow lesions are subcategorized as capillary, lymphatic, and venous.(1) Capillary malformations are flat, sharply demarcated, red-pink vascular stains of the skin commonly referred to as port-wine stains. These persist throughout life and are characterized histologically by dilated capillaries within the dermis. They slowly increase in size with age. Lymphatic malformations are spongelike collections of abnormal channels and spaces that contain clear lymphatic fluid, causing an excess of fluid to accumulate and dilate the lymphatic channels. This results in swelling of the affected area and, if extensive, can cause enlargement of soft tissues and bones.

Preferred methods of excess hair removal in pediatric patients with lung transplantation.

BACKGROUND: Hypertrichosis is common among recipients of lung transplantation taking cyclosporine and it poses a special psychosocial problem in children. Children and parents frequently inquire about methods of hair removal. OBJECTIVE: We sought to determine preferred methods of hair removal in this population. METHODS: We distributed a questionnaire to 104 pediatric recipients of lung transplantation. A total of 56 questionnaires (54%) were completed. RESULTS: Of respondents, 91% (51 of 56) experienced hypertrichosis, 75% (38 of 51) of whom attempted treatment. Depilatory cream was the most common hair removal technique (79%, 30 of 38). Shaving, particularly with an electric razor, received the highest ratings of both satisfaction and likelihood of future use. LIMITATIONS: Generalizability is limited by the response rate. Preferred methods according to different hair locations were not evaluated. CONCLUSION: In our pediatric transplantation population, depilatory cream was the most commonly used treatment for hypertrichosis whereas electric shaving had the highest ratings of satisfaction and future use.

Inflammatory tinea pedis/manuum masquerading as bacterial cellulitis.

BACKGROUND: Tinea pedis and tinea manuum in children are more common than previously recognized. Clinical presentations of dermatophyte infections may vary in children and may be difficult to diagnose. OBJECTIVE: To show the necessity of potassium hydroxide preparations and/or fungal cultures in assessing suspicious cases of cellulitis in children who may have dermatophyte infections. PATIENTS: We describe 4 children with inflammatory tinea pedis or tinea manuum who were initially misdiagnosed as having bacterial cellulitis. INTERVENTION: A potassium hydroxide examination was performed on 3 patients. Fungal cultures were performed on 2 patients. RESULTS: Inflammatory/bullous dermatophyte infections were detected by potassium hydroxide examination in all 4 patients and all 4 children successfully responded to topical antifungal therapy. CONCLUSIONS: These cases demonstrate that inflammatory tinnea pedis/manuum can masquerade as cellulitis in children. Early potassium hydroxide examination can allow appropriate antifungal treatment to be initiated before fungal culture results are finalized.

Cutaneous manifestations of hyper-IgE syndrome in infants and children.

We describe 8 children with hyper-IgE syndrome who had papulopustular eruption on the face and scalp in the first year of life. Seven of the 8 patients had persistent peripheral eosinophilia and 3 had leukocytosis noted before diagnosis. Skin biopsy specimens in 6 patients revealed spongiosis and perivascular dermatitis and/or folliculitis with a predominance of eosinophils. Two patients had bone fractures and osteopenia. Recurrent pneumonia occurred in 6 children and pneumatoceles in 5. The diagnosis of hyper-IgE syndrome was made an average of 18 months after the onset of the initial papulopustular eruption. These findings may lead to earlier recognition of the disease and institution of appropriate treatment.

Nevoid basal cell carcinoma syndrome in an African American woman.

Nevoid basal cell carcinoma syndrome (NBCCS) is uncommon in African American patients. Also, basal cell carcinomas (BCCs) arise less frequently in African American patients with NBCCS than in white patients. We present a case of an African American woman with NBCCS.