Juvenile pemphigus vegetans of the glans penis.

Abstract

Sir, Pemphigus is defined as a group of mucocutaneous blistering diseases characterized by epithelial acantholysis. In all variants of pemphigus, circulating and fixed autoantibodies against the intercellular substance of epithelium have been demonstrated. Suprabasal clefting is seen in pemphigus vulgaris and its rare variant, pemphigus vegetans (1). The latter form of pemphigus is characterized by papillomatous and vegetative lesions and accounts for about 5% of cases. It is historically differentiated into two types (2). In the Neumann type, often beginning as pemphigus vulgaris, soft whitish vesicles soon erode and heal with verrucous hypertrophic growths. In the Hallopeau type, yellowish pustules are the primary lesions; crusted, verruciform or papillomatous proliferations then develop with an advancing border of new pustules (1). The course of disease is relatively benign (3). Both entities affect primarily the intertriginous areas (4). In addition, broken bullae and erosions with vegetations may occur on the oral, nasal or oesophageal mucosa (5 – 7); the involvement of the genital mucosa is exceedingly rare. Herein we report the occurrence of pemphigus vegetans of the Neumann type with both cutaneous and mucous manifestations in a young boy.