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


CONCLUSIONS
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.
Nigen
Simon
S
Department of Dermatology, Centre Hospitalier Universitaire de Québec, pavillon Hotel-Dieu de Québec, Québec, Québec, Canada.
Poulin
Yves
Y
Rochette
Linda
L
Lévesque
Marie-Hélène
MH
Gagné
Eric
E
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008875:Middle Aged; D011711:Pyoderma / Q000175:diagnosis* / Q000188:drug therapy; D013280:Stomatitis / Q000175:diagnosis* / Q000188:drug therapy
D000305:Adrenal Cortex Hormones