Pyostomatitis vegetans in childhood

Abstract

Pyostomatitis vegetans is an oral eruption, characterized by small pustules, ulcers and erythematous vegetations of the labial and buccal mucosae as well as labial-attached gingivae. Its importance lies in its high correlation with inflammatory bowel disease. It is commonly associated with skin and inflammatory bowel disease and is rare in children. We here report a sister and brother with onset of the disease at the age of 5 and 7 years, respectively. It is the first report of familial pyostomatitis vegetans occurring in the youngest patients hitherto reported.

Conclusion: The observation of two sibs with pyostomatitis, vegetans pyoderma gangrenosum and inflammatory bowel disease suggest a hereditary disposition to this rare triad.