Pyostomatitis vegetans (PSV) is a rare inflammatory disorder that is often associated with underlying inflammatory bowel disease (IBD [ie, ulcerative colitis, Crohn disease]) and less commonly with primary sclerosing cholangitis. Men are more frequently affected than women, and patients are usually aged 20-60 years when diagnosed. The onset of bowel disease can precede this disease by years. PSV may also be a marker of subclinical disease. Mucosal manifestations, predominantly oral, are characteristic. About half of patients develop cutaneous lesions as well. The term pyostomatitis-pyodermatitis vegetans (PSV-PDV) is used in cases of mucocutaneous disease. The pathogenesis of PSV and PDV is poorly understood.