National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Orofacial Granulomatosis

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.[1][2] Up to 40% of the people with orofacial granulomatosis (OFG) may have positive reactions to patch allergy tests.[3]

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.[4][3]  Granulomatous cheilitis or OFG may improve with a cinnamon- and benzoate-free diet.[3]  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.[1]
Last updated: 9/5/2018

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet New Zealand is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Primary Care Dermatology Society has information on this disease. Please click on the link. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Orofacial Granulomatosis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Dyall-Smith. Orofacial granulomatosis. DermNetNZ. 2016; http://www.dermnetnz.org/topics/orofacial-granulomatosis/.
  2. Rana AP. Orofacial granulomatosis: A case report with review of literature. Journal of Indian Society of Periodontology Available at:. 2012; 16(3):469-474. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3498725/.
  3. Snyder A. Cheilitis Granulomatosa Treatment & Management Updated. Medscape Reference. Jun 21, 2018; https://emedicine.medscape.com/article/1075333-treatment.
  4. Al-Hamad A, Porter S & Fedele S. Orofacial Granulomatosis. Dermatol Clin. July, 2015; 33(3):433-46. http://www.ncbi.nlm.nih.gov/pubmed/26143423.
  5. Troiano G, Dioguardi M, Giannatempo G, et al. Orofacial Granulomatosis: Clinical Signs of Different Pathologies. Medical Principles and Practice. 2015; 24(2):117-122. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5588207/.