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.

Treatment of toxic epidermal necrolysis in a pediatric patient with a nanocrystalline silver dressing.

Background/purpose: To report a case of toxic epidermal necrolysis (TEN) involving 90% body surface area, successfully treated with a nanocrystalline silver dressing (Acticoat, Smith & Nephew, Largo, FL).

Methods: A review of the hospital and acute wound center patient records and the recent English medical literature regarding TEN and nanocrystalline silver dressing.

Results: We found only 1 report of TEN treated with a nanocrystalline silver dressing.

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.

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.

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.

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.

Mutations in ABCA12 underlie the severe congenital skin disease harlequin ichthyosis.

Harlequin ichthyosis (HI) is the most severe and frequently lethal form of recessive congenital ichthyosis. Although defects in lipid transport, protein phosphatase activity, and differentiation have been described, the genetic basis underlying the clinical and cellular phenotypes of HI has yet to be determined. By use of single-nucleotide-polymorphism chip technology and homozygosity mapping, a common region of homozygosity was observed in five patients with HI in the chromosomal region 2q35.

Congenital calcinosis cutis of the ear.

Congenital calcinosis cutis is a relatively rare event. Herein we report 3 cases of congenital calcinosis cutis that all appeared in the exact same location on the ear. A possible mechanism of pathogenesis is discussed.

Loss of kindlin-1, a human homolog of the Caenorhabditis elegans actin-extracellular-matrix linker protein UNC-112, causes Kindler syndrome.

Kindler syndrome is an autosomal recessive disorder characterized by neonatal blistering, sun sensitivity, atrophy, abnormal pigmentation, and fragility of the skin. Linkage and homozygosity analysis in an isolated Panamanian cohort and in additional inbred families mapped the gene to 20p12.3.

Fluocinolone acetonide 0.01% in peanut oil: therapy for childhood atopic dermatitis, even in patients who are peanut sensitive.

Background: Fluocinolone acetonide 0.01% in a blend of refined peanut and mineral oils has been used as treatment for scalp psoriasis for several years, but only more recently for atopic dermatitis.

Objective: We sought to study the effectiveness for atopic dermatitis, potential for adrenal axis suppression, and safety of the fluocinolone acetonide 0.

Hypertrichosis.

Hypertrichosis is hair growth that is abnormal for the age, sex, or race of an individual, or for a particular area of the body. Recognized forms of hypertrichosis are reviewed. Hirsutism, which is male-pattern hair growth in a female or child, is not included in this review.

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.

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.

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.

Diaper dermatitis.

Preview Skin eruptions in the diaper area in infants bring many new parents to the primary care physician's office. Which topical agents and diapering strategies work best to clear lesions? And how can recurrence be avoided? The authors answer these questions and discuss common skin disorders in the diaper region as well as several rare disorders that may require dermatologic consultation.