Lawrence A. Schachner

Childhood bullous pemphigoid. Clinical and immunologic features, treatment, and prognosis.

A 2 1/2-month-old female infant presented with multiple tense bullae on the hands and feet. Analysis of biopsy specimens confirmed our clinical impression of childhood bullous pemphigoid. Confirmatory data included type IV collagen mapping of the basement membrane zone, a readily available technique that helps distinguish childhood bullous pemphigoid from childhood epidermolysis bullosa acquisita. To our knowledge, our patient is the youngest described with childhood bullous pemphigoid, and we use this opportunity to review the literature and examine the clinical and immunologic features, treatment, and prognosis of this rare childhood immunobullous disorder.

Isotretinoin treatment of Darier's disease

A total of 104 patients with Dariers disease were treated with 13- cis -retinoic acid (isotretinoin) at nineteen medical centers. Clinical evaluation by both physicians and patients indicated that the drug is very effective in alleviating Dariers disease but does not produce prolonged remissions.

A clinical trial comparing the safety and efficacy of a topical erythromycin-zinc formulation with a topical clindamycin formulation

One hundred three patients with acne vulgaris were randomly designated to receive either a topical formulation of erythromycin plus zinc or a topical solution of 1% clindamycin phosphate (Cleocin-T). The patients treated themselves twice daily and were examined at 3, 6, 9, and 12 weeks after the start of therapy. By week 6 the overall severity grade was consistently lower and the percent reduction of severity, papules, pustules, and total comedones was higher in the erythromycin-zinc-treated group than in the clindamycin-treated group. In the 92 patients who completed this study (48 receiving erythromycin-zinc and 44 receiving clindamycin), no serious topical or systemic side effects were reported. Two patients, one from each treatment group, suffered mild irritation. One patient was withdrawn from the erythromycin-zinc-treated group. Results of patch tests were negative. The superiority of the erythromycin-zinc formulation may be due to the increased (4%) erythromycin concentration and/or the ability of 1.2% zinc acetate to enhance the products activity.

Giant Aplasia Cutis Congenita without Associated Anomalies

Abstract:  Aplasia cutis congenita is a congenital condition in which skin, bone, and dura can be absent. The condition can present in isolation or with associated conditions such as limb anomalies or embryologic malformations. The majority of cases affect the scalp and are limited to the dermis and epidermis. Vertex aplasia cutis typically range in size from 0.5 to 3 cm. The rare larger scalp defects are prone to complications of hemorrhage and infection, and subsequently patients are at risk for death. For these reasons, surgical intervention for large defects may be required. We report the case of a 12‐month‐old Haitian boy who presented with aplasia cutis congenita of the scalp involving 10 cm of skin and 9 cm of underlying bone. There were no other associated anomalies.

Sézary syndrome in an 11-year-old girl

All forms of cutaneous T-cell lymphoma are rare in children. We describe an 11-year-old girl who had generalized exfoliative erythroderma, intense pruritus, peripheral lymphadenopathy, mycosis cells in the skin and lymph nodes, and Sézary cells in the peripheral blood. Results of a biopsy specimen of involved skin showed changes consistent with mycosis fungoides. A classic case of Sézary syndrome has not previously been reported in childhood or preadolescence.

Topical erythromycin and zinc therapy for acne

A double-blind, 12-week study was undertaken to determine the safety and efficacy of a formulation of 4% erythromycin plus 1.2% zinc acetate compared with its vehicle. The study was continued for 40 weeks after the 12-week double-blind phase by switching vehicle-treated patients to active treatment and continuing to give patients treated with active drug the same treatment. Seventy-three female patients started the study; 39 completed 1 full year of study. In the first 12 weeks statistically significant differences were noted in the efficacy of the erythromycin-zinc compared with vehicle for acne severity grades (global assessment) and for papule, pustule, and comedo counts. After crossover, the vehicle-treated group receiving active therapy duplicated the improvement of the group initially treated with erythromycin-zinc. No clinical problems with superinfection or secondary infection occurred during 1 year of treatment in 39 patients.

Acquired progressive lymphangioma

Acquired progressive lymphangioma is rare and can histologically mimic well-differentiated angioendothelioma. In a 9-year-old boy with acquired progressive lymphangioma, we demonstrated that the neoplasm consists of at least a vascular component and a smooth muscle component. The presence of type IV collagen around many vascular channels suggests that an intermediate stage of differentiation between blood vessels and lymphatics may be present. Our findings indicate that acquired progressive lymphangioma is most likely a complex hamartoma composed of vascular channels and smooth muscle.

Diagnosis and Treatment of Childhood Scabies and Pediculosis

Human scabies infestations are still a widespread occurrence. This article discusses the clinical features, infections, lesions, and sites of involvement of different types of scabies as well as pediculosis.

Blau syndrome: A new kindred

Blau syndrome is a rare condition typically defined by granulomatous arthritis, skin eruption, and uveitis occurring in the absence of lung or other visceral involvement. Other characteristic physical findings include synovial cysts and camptodactyly. We describe a new kindred demonstrating autosomal dominant inheritance and anticipation.

Eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis was first described by Ofuji et al in 1970. It is characterized by pruritic circinate plaques that are studded with follicular papules and pustules. Lesions are located chiefly on the face, trunk, and arms. Biopsies of lesions demonstrate an infiltrate of eosinophils and neutrophils within hair follicles, dermis, and epidermis. Peripheral leukocytosis and eosinophilia are common.