The neumann type of pemphigus vegetans treated with combination of dapsone and steroid

Abstract

Pemphigus vegetans is a rare variant of pemphigus vulgaris and is characterized by vegetating lesions in the inguinal folds and mouth and by the presence of autoantibodies against desmoglein 3. Two clinical subtypes of pemphigus vegetans exist, which are initially characterized by flaccid bullae and erosions (the Neumann subtype) or pustules (the Hallopeau subtype). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery of the lesions. Oral administration of corticosteroids alone does not always induce disease remission in patients with pemphigus vegetans. We report here on a 63-year-old woman with pemphigs vegetans. She had a 2-year history of vegetating, papillomatous plaques on the inguinal folds and erosions of the oral mucosa. The enzyme-linked immunosorbent assay was positive for anti-desmoglein 3, but it was negative for anti-desmoglein 1. She was initially treated with systemic steroid, but no improvement was observed. The patient was then successfully treated with a combination of systemic steroid and dapsone with a good clinical response.

Keywords: Dapsone; Pemphigus vegetans.

Fig. 1

Oozing erosive vesicles on the lip (A) and hypertrophic verrucous vegetative plaques on the inguinal folds (B).

Fig. 2

(A) A biopsy of an erosive vesicle from the lip showed a suprabasal cleft with scattered acantholytic cells (H&E, ×20). (B, C) A biopsy of the vegetating plaque from the inguinal folds showed a hyperplastic epidermis, eosinophilic spongiosis, and intraepithelial abscesses packed with eosinophils. Eosinophils also dominated the dense inflammatory infiltrate in the papillary dermis (H&E, ×10, ×20).

Fig. 3

(A, B) The lesion improved and disappeared 3 weeks after treatment.