Pemphigus vegetans presenting as serpiginous oral, esophageal and genital mucosal ulcers undiagnosed for 3 y

Abstract

Pemphigus vegetans (PVeg) is a relatively milder and chronic variant of pemphigus vulgaris. It is very rare, constituting only about 3% of all cases of pemphigus. It is characterized by initial bullae or pustules which may then progress to papillomatous vegetations or plaques. Usually, oral mucosal and cutaneous lesions are seen in the same patient. Very rarely, only mucosal presentation may be seen. In this report, we present a case of a 30‑year‑old woman with previously undiagnosed multiple, painful, chronic vegetative ulcers with “snail‑track”‑like appearance present for 3 years. Extensive clinical and laboratory investigations revealed the lesions to be due to PVeg. The patient responded to prednisolone and cyclophosphamide therapy with complete remission after 2 months.

Keywords: Mucosa, pemphigus vegetans, serpiginous, ulcers, vegetations