This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
Orofacial granulomatosis (OFG) is a condition characterized by granulomatous inflammation of regions of the mouth, jaw and face (maxillofacial), in the absence of a recognised systemic condition known to cause granulomas. Features include lip enlargement, swelling inside and around the mouth, oral ulcerations (sores), and inflammation of the gums (gingivitis). There may be only swelling inside the mouth or permanent disfiguring swelling of the lips and face. OFG includes granulomatous cheilitis (when it presents as a persistent or recurrent lip swelling), and Melkersson-Rosenthal syndrome (which includes CG, facial nerve palsy and fissured tongue) that can manifest with only CG. In some cases, orofacial granulomatosis is part of another disease such as Crohn’s disease, sarcoidosis, and infectious diseases such as tuberculosis. The diagnosis of OFG is confirmed only by biopsy and microscopic tissue analysis identifying the noncaseating granulomas. When OFG occurs alone, without other associated diseases it is considered idiopathic. Up to 40% of the people with orofacial granulomatosis (OFG) may have positive reactions to patch allergy tests.
The exact prevalence of idiopathic OFG is not known, but it is considered a rare condition in most literature reports. Treatment is difficult and has to be individualized, but may include corticosteroids (systemic or injected inside the lesions), and other medication. Granulomatous cheilitis or OFG may improve with a cinnamon- and benzoate-free diet. Response to treatment is slow, and can take years, but most people improve. Surgery may be required for severe permanent swelling interfering with speaking or eating.
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