Childhood bullous pemphigoid

Abstract

A two-year-old girl was presented with vesiculobullous eruptions initially on the palmoplantar region then extend to the whole body (Figure 1). On examination multiple tense vesicles, bullae and crusted erosions touching the whole tegument were seen. Nikolsky’s sign was negative. Mucous membrane examination revealed erosions over buccal mucosa and hard palate. There was no regional lymphadenopathy. Histopathological examination revealed subepidermal blister and a mild inflammatory infiltrate on the dermis. Direct immunofluorescence (DIF) showed linear deposition of IgG and C3 at the dermoepidermal junction. The specific blood test searching for cutaneous basement membrane antibodies was positive. Based on clinical, histological and immunopathological criteria, the diagnosis of childhood bullous pemphigoid was made. The patient was treated with prednisone 1 mg/kg/ day associated with methotrexate 5 mg/week which led to a rapid resolution of the skin lesions within two months. Topical steroids and dapsone were ineffective in this case.