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.