Case for diagnosis.

Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis characterized by pustular and vegetating lesions of the skin and oral mucosa. It is considered a highly specific marker for inflammatory bowel diseases. The authors describe a case of pyodermatitis-pyostomatitis vegetans in a pediatric patient who presented marked clinical improvement after beginning treatment with oral corticosteroids, azathioprine, and dapsone. Bowel surveillance is mandatory, since the dermatosis is associated with inflammatory bowel diseases in more than 70% of patients, especially ulcerative colitis.