Orofacial Granulomatosis: Food for Thought!

Abstract

Orofacial granulomatosis is a relatively recent term coined by Weinsfeld et al in 1995 to describe the presence of oral granulomatous lesions without intestinal involvement. It could manifest either as a triad involving facial nerve, lip swelling, fissured or furrowed tongue referred to as Melkerson Rosenthal Syndrome (MRS) or its monosymptomatic or oligosymptomatic forms referred to as Granulomatous Cheilitis (GC). 31-year-old Caucasian male with complaints of painful swelling of the lips with recurrent oral ulcers and difficulty eating for 3 years. Exam was significant for painful, right-sided lip, tongue, and facial swelling with an 8 mm shallow intraoral ulcer. ESR, quantiferon tb, celiac panel, ACE levels, heavy metal and food allergen test were negative ASCA antibodies were positive but EGD, CT- enterography and colonoscopy which showed no evidence of intestinal involvement. Biopsy of oral lesion revealed multiple non-caseating granulomas with multinucleated giant cells consistent with granulomatous cheilitis. Patient did not respond to dapsone, oral and intralesional corticosteroids, so infliximab 10 mg/kg induction dose followed by 10 mg/kg every 4 weeks was started with resolution of the swelling and ulcers. Granulomatous cheilitis is a persistent, relapsing remitting, idiopathic, non-tender swelling of one or both lips. The etiology is unknown, however relationship to crohn's disease, sarcoidosis and various infectious and allergens has been reported but none has been validated. There is a distinct clinical entity backed by genetic studies which have suggested that OFG may have a different HLA profile than Crohns disease. Topical, systemic and intralesional corticosteroids have been used with variable outcomes. Infliximab was first reported for the treatment of orofacial Crohns in 2001.There have been 5 other case reports that have described the use of Infliximab or adalimumab for the treatment of OFG. The dose of Infliximab used in most of those studies was 3-5 mg/kg and the duration of treatment varied from 3 weeks to 21 month. Our patient had resolution of lesions with 10 mg/kg of infliximab and has had sustained response for the past 8 months. In conclusion, we believe that OFG is a distinict entity seperate from Crohns disease and high dose Infliximab should be considered an alternative treatment options in patients who do not respond to other treatment regimens.