pemphigoid vegetans in childhood: a case report and short review of literature

Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions.