Oral Crohn's disease and pyostomatitis vegetans: An unusual association

Abstract

Oral features of Crohn's disease include ulcers, lip fissuring, cobblestone plaques, angular cheilitis, polypoid lesions, and perioral erythema. Pyostomatitis vegetans is a rare eruption of the oral mucosa characterized by tiny yellow pustules. It is considered a marker for inflammatory bowel disease. We describe a 45-year-old woman with a 6-month history of painful sores in her mouth, diarrhea, weight loss, and cutaneous lesions. Oral examination revealed cobblestone plaques and indentation on the tongue and friable vegetating pustules on the labial commissures. Staphylococcus simulans was isolated from the pustules. Laboratory studies revealed leucocytosis, eosinophilia, and low hemoglobin and zinc levels. Histologic study of the labial lesions revealed hyperplastic epithelium with intraepithelial clefts that contain eosinophils and neutrophils. Tongue lesions showed chronic inflammation with noncaseating granulomas. Later, colonoscopy and biopsy demonstrated Crohn's disease of the anorectal region. Pyostomatitis vegetans lesions regressed after oral zinc supplementation. Prednisone treatment resulted in healing of the tongue lesions. In our patient, pyostomatitis vegetans appeared to be related to zinc deficiency that may have been caused by malabsorption. The pathogenetic interrelationship between pyostomatitis vegetans and Crohn's disease is discussed.