Update on Orofacial Granulomatosis

Orofacial granulomatosis (OFG) is a condition manifesting clinically with chronic swelling of the mouth and/or face, notably with swelling of the lips and oral mucosa, a full-thickness, erythematous gingivitis and mucosal ulceration of various clinical types. Some patients may also present with neurological findings, for example facial palsy. Biopsy of affected tissue shows lymphoedema, with or without granulomatous inflammation. The oral lesions in OFG are histologically indistinguishable from the oral lesions in Crohn's disease (CD) and other systemic granulomatous disorders. It is a condition which may respond to the exclusion of certain food-related chemicals from the diet in up to 60% of patients and, as such, is distinct from gastrointestinal CD. CD is a relapsing systemic inflammatory disease which predominantly affects the gut, and patients suffering from this disease frequently present with abdominal pain, fever and altered bowel habit. A proportion of patients with clinical OFG (without other systemic disease) may have asymptomatic gastrointestinal involvement or go on to develop gut CD suggesting an association between the two diseases. It is estimated that 1% of CD sufferers may have a diagnosis of OFG, but the majority of patients in specialist OFG clinics do not have gut symptoms.