Orofacial granulomatosis in childhood-a clinical entity that may indicate Crohn's disease as well as food allergy

Orofacial granulomatosis (OFG) is a rare clinical entity with orofacial swelling in association with oral lesions such as mucosal oedema, ulcerations and mucosal tags. The aim of this prospective study was to evaluate the connection between OFG in childhood and systemic disease. During a 3-year period, eight children (9-16 years old) who had been referred to the clinic of oral medicine were diagnosed solely with OFG. Thus, none of them had any known systemic disease or gastrointestinal symptoms at the time of referral. The children were then medically examined and followed up for 6-8 years at the department of paediatrics for systemic disease with specific emphasis on inflammatory disorders elsewhere in the gastrointestinal tract. During follow-up, four patients were diagnosed with Crohn's disease (CD). Further, one girl was found to have a food allergy-induced OFG, with delayed-type hypersensitivity to oats. One boy developed both diabetes and celiac disease during the follow-up. Only two patients had no diagnosis of systemic disease at the end of the observation period. OFG in childhood seems to be frequently related to systemic disease, and children with OFG should be referred to a paediatrician for examination and follow-up.