title: 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.
  
  
  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.
authors.affiliation: Department of Dermatology, Centre Hospitalier Universitaire de Québec, pavillon Hotel-Dieu de Québec, Québec, Québec, Canada.
authors.forename: Simon
authors.initials: S
authors.lastname: Nigen
authors.forename: Yves
authors.initials: Y
authors.lastname: Poulin
authors.forename: Linda
authors.initials: L
authors.lastname: Rochette
authors.forename: Marie-Hélène
authors.initials: MH
authors.lastname: Lévesque
authors.forename: Eric
authors.initials: E
authors.lastname: Gagné
mesh_terms: 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
chemical_list: D000305:Adrenal Cortex Hormones