Childhood bullous pemphigoid treated by i.v. immunoglobulin

Abstract

Bullous pemphigoid is an acquired autoimmune subepidermal blistering disorder mostly seen in the elderly. Childhood bullous pemphigoid is very rare. For the first time we report a case of childhood bullous pemphigoid associated with infantile eczema. Two weeks after a routine vaccination, a 3.5-month-old boy with infantile eczema developed a generalized blistering disorder. Histopathology revealed a subepidermal blister. Direct immunofluorescence showed linear depositions of C3 along the basement membrane zone. Indirect immunofluorescence studies demonstrated the presence of circulating immunoglobulin G antibodies directed against the epidermal side of salt-split skin. Enzyme-linked immunosorbent assay demonstrated serum level of anti-BP180 antibody elevated. The patient was successfully treated by high-dose i.v. immunoglobulin.

Infantile Bullous Pemphigoid Treated Using Intravenous Immunoglobulin: Case Report and Review of the Literature

“…Although IBP usually responds well to first‐line therapy with systemic corticosteroids, resistant cases have been reported. A literature search revealed anecdotal cases of IBP treated using IVIG (Table ) . Variable dosages (200 mg/kg/day to 1 g/kg/day), infusion courses (2‐ to 5‐day courses), and intervals (2–5 wks) were used, probably reflecting the heterogeneity of institutional or individual preferences regarding IVIG treatment in general.…”

Childhood Bullous Pemphigoid: A Case Report and Literature Review

“…The lesions of childhood BP are large, tense bullae with diffuse distribution, affecting the inner side of the thigh, forearms, axillae, lower part of the abdomen, groin, palms and soles. Diagnosis is based on clinical, histological, immunopathological features [1][2][3].…”

“…Direct immunofluorescence (DIF) of skin biopsy of unblistered, perilesional skin (normal-looking skin) reveals linear deposition of IgG and/or complement 3(C3) along the basement membrane zone (BMZ). Indirect immunofluorescence (IIF) shows presence of circulating IgG anti-basement membrane zone antibodies [1][2][3][4]. We report a case of childhood bullous pemphigoid whose skin biopsy showed subepidermal blisters with dermal layer infiltrated with neutrophils.…”

Intravenous Immunoglobulin Therapy in Dermatologic Disorders

“…Clearing of all lesions was observed and corticosteroid therapy could be discontinued after a mean of 3.3 months [25]. Also a 3.5-month-old child with childhood bullous pemphigoid was successfully treated with 400 mg/kg/day IVIG, for 4 days [27].…”