Pemphigus vegetans: A case report and review of the literature
Keywords:
pemphigus vegetans, vesiculobulous diseaseAbstract
Pemphigus vegetans is a very rare variant of pemphigus vulgaris, which is an autoimmune blistering disease of the skin and mucosal membrane. Pemphigus vegetans initially manifests as pustules or vesicles but these primary lesions subsequently develop into vegetating plaques. Typically, these are located in intertriginous areas and the oral mucosa. The histopathological findings of pemphigus vegetans are suprabasal acanthosis, epidermal hyperplasia, papillomatosis, and intraepidermal eosinophilic abscess. Immunofluorescence tests results are indistinguishable from those of pemphigus vulgaris. Pemphigus vegetans has been divided into 2 clinical subtypes, the Neumann type and the Hallopeau type. The Neumann type has a similar course to pemphigus vulgaris, and requires treatment with a high dose of corticosteroids. The Hallopeau type, by comparison, has a more benign course. This report describes a case of pemphigus vegetans Neumann type where the patient presented with typical manifestations and responded to treatment with corticosteroids alone.
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