Pyostomatitis Vegetans With Orofacial and Vulvar Granulomatosis in a Pediatric Patient

Pyostomatitis vegetans (PSV) is a rare disorder strongly associated with inflammatory bowel disease (IBD), most commonly ulcerative colitis, but has infrequently been found in patients with Crohn disease (CD). We present the case of a 7-year-old girl with PSV and extraintestinal findings suggestive of CD-chelitis granulomatosa, perianal and vulvar edema with biopsies revealing noncaseating granulomas, and anal skin tags-and an elevated calprotectin noted during a cutaneous flare. She did not have clinical or endoscopically identified underlying gastrointestinal involvement for 4 years after symptom onset. Given the paucity of data on managing PSV in a child without gastrointestinal findings of IBD, the literature is reviewed and treatment options discussed.