Pyodermatitis-pyostomatitis vegetans: two cases and a review of the literature

Abstract

Background: Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare vegetating, pustular, eosinophilic, mucocutaneous dermatosis characterized by mucocutaneous lesions of the genital, axillary, and oral regions, as well as on the scalp.

Objective: We report two patients who were diagnosed with PD-PSV. The published cases of this rare disorder are reviewed.

Results: The first patient presented with vegetating pustular plaques of the scalp, vulva, and mouth. The second patient initially showed pustules on fingers and then on toes. Vulva and mouth were later involved. There was no gastrointestinal involvement in either case. In both cases, histology revealed eosinophilic spongiosis with eosinophilic microabscesses and pseudoepitheliomatous hyperplasia. Direct immunofluorescence was negative.

Conclusion: Pyodermatitis-pyostomatitis vegetans is a rare disorder of unknown cause. It bears similarities to pemphigus vegetans but histology, direct immunofluorescence, and clinical history show the differences. Response to treatment is generally rapid. In several reported cases, an inflammatory gastrointestinal disorder is found in association with the mucocutaneous signs.