PYOSTOMATITIS-PYODERMATITIS VEGETANS REVE ALING CROHNS-DISEASE

Background. Pyostomatitis-pyodermatitis vegetans is an uncommon condit ion associated with chronic inflammatory bowel disease in 75% of the c ases, usually hemorrhagic rectocolitis. Case report. A 48-year-old man was referred for recent development of pustulous lesions of the lips and buccal mucosa and weight loss. He complained of abdominal pain and intermittent diarrhea which had persisted for more than one year. Dur ing the last three months, a pseudotumoral plaque with a pustulous rim had developed over the two distal phalanxes of the right middle finge r in association with ungueal lysis and nodular, vegetating and cruste d lesions on the lateral aspect of the left arm. Small pustules covere d the entire buccal mucosa excepting the tongue and the glans forming a typical snail trace aspect. Bacterial and mycological samples were n egative. The histology reports for skin and mucosa were similar: epith elial hyperplasia, intra-and subepithelial granulocyte micro-abscesses and polymorphous infiltration of the superficial derma with numerous neutrophils and eosinophils. There was a discrete interkeratinocytic f luorescence at direct immunofluorescence but indirect immunofluorescen ce was negative. Anti-desmogleine 1 immunolabeling showed typical norm al skin uptake and immunotransfer was negative. Digestive tract endosc opy and histopathology examination of the bowel specimens confirmed th e diagnosis of Crohn's disease. Clinical manifestations improved drama tically with prednisone. Discussion. This case of pyostomatitis-pyoder matitis vegetans involved several aspects rarely reported in the liter ature: a) the cutaneomucosal signs were inaugural; b) the association with Crohn's disease; c) the presence of lesions to the genital mucosa ; d) the unusual localization of the inaugural skin manifestations. Th is clinical entity has now been clearly distinguished from pemphigus v egetans. There was however a long debate on the similar clinical, hist ological and even immunological expressions. We suggest that pyostomat itis-pyodermatitis vegetans belongs to the spectrum of neutrophilic de rmatoses and other authors even propose it is a clinical form of pyode rma gangrenosum.