S2706 Orofacial Granulomatosis in Crohn's Disease

Abstract

Introduction: Extra-intestinal manifestations (EIMs) of inflammatory bowel disease (IBD) are rare complications that affect roughly 6-25% of IBD patients. Orofacial granulomatosis (OFG) is a rare manifestation that does not have reliable epidemiologic data and is characterized by orofacial swelling with non-caseating granulomas seen on pathology. Case Description/Methods: A 23-year-old male with a history of Crohn’s colitis presented with loose stools and hematochezia for 7 months and 1 year of left sided lip swelling. He was diagnosed with Crohn’s disease at age 15 and was maintained on oral mesalamine and 6-mercaptopurine (6-MP). At age 16, the patient was evaluated for supraglottic edema. Urgent tonsillectomy was performed, with pathology revealing necrotizing and non-caseating granulomas. He recovered without complication. He achieved endoscopic remission at age 18 and his medications were discontinued. Unfortunately, he was lost to follow-up. During this subsequent presentation, his vitals were within normal limits. Exam was notable for left-sided lip and cheek swelling without mass or lesion. Bloodwork was notable for an ESR of 19 and CRP of 7.5. Lip biopsy showed normal squamous epithelium and non-caseating granulomas, consistent with OFG. Patient was treated for a Crohn’s flare with oral prednisone as a bridge to 6-MP. His OFG was treated with intralesional corticosteroids without relief and has on-going outpatient follow up. His current treatment plan includes further cycles of steroid injections before considering alternative therapies. His most recent colonoscopy showed endoscopic and histologic remission of his luminal Crohn’s while on infliximab. (Figure) Discussion: OFG is frequently seen as a manifestation of a systemic condition such as IBD. OFG is diagnosed by biopsy and clinical suspicion. OFG specific treatments include topical steroids and calcineurin inhibitors. Recent data suggests that a cinnamon/benzoate free diet may provide modest benefit to patients. Adequate treatment of underlying IBD is equally important. This patient has the classic symptoms and histopathologic findings of OFG and likely had undiagnosed OFG for many years given his tonsillar pathology years prior. Despite treatment, this patient has persistent and refractory symptoms. This case highlights a classic presentation of a rare IBD extra-intestinal manifestation and demonstrates how EIMs can occur even with well controlled IBD.Figure 1.: Orofacial Granulomatosis: Lip and Cheek Swelling.