Approach Considerations

Laboratory studies

Serum angiotensin-converting enzyme (SACE) testing may be performed to help exclude sarcoidosis. Patch tests may be used to help exclude reactions to metals, food additives, or other oral antigens^[35]; some cases of granulomatous cheilitis may be associated with such sensitivities. If found, avoidance of the implicated allergen is recommended. Decreased iron, hemoglobin, ferritin, folate, and elevated C-reactive protein, celiac antibodies, serum IgE, and alkaline phosphatases have been documented to be associated with orofacial granulomatosis (OFG); however, evidence is insufficient that any of these are consistent markers of the disease.^[5]

Imaging studies

OFG is the term given to granulomatous lesions similar to those of Crohn disease, found on oral biopsy, but where there is no detectable systemic Crohn disease, though this may be detected later.^[36]Gastrointestinal tract endoscopy, radiography, and biopsy may be used to help exclude Crohn disease. Chest radiography or gallium or positron emission tomography (PET) scanning may be performed to help exclude sarcoidosis and tuberculosis. Panorex dental films may be obtained to assess for the presence of a chronic dental abscess.

Procedures

A biopsy of the swollen lip or orofacial tissues is indicated but often shows only lymphoedema and perivascular lymphocytic infiltration during the early stages and may only later show granulomas. A biopsy may help to exclude Crohn disease, sarcoidosis, lymphoma, and other conditions in the differential diagnosis.

Histologic Findings

Histologic changes are not always conspicuous or specific in many cases of long duration; the infiltrate becomes denser and pleomorphic, and small, focal, noncaseating, sarcoidal granulomas are formed that are indistinguishable from Crohn disease or sarcoidosis.

The inflammatory response is probably mediated by cytokines such as tumor necrosis factor-alpha and by protease-activated receptors (PARs), matrix metalloproteinases (MMPs), and cyclo-oxygenases (COXs). There is submucosal chronic inflammation with many Th1 and mononuclear, interleukin 1–producing cells; large, active, dendritic B cells; and noncaseating granulomas.^[37]Small granulomas occur in the lymphatic walls in some cases. Similar changes may be present in cervical lymph nodes.

Treatment & Management

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Media Gallery

Orofacial granulomatosis in a patient with Crohn disease showing showing lip and gingival swelling.

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Author

Alan Snyder MD Candidate, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Crispian Scully, MD, MRCS, PhD, MDS, CBE, FDSRCS(Eng), FDSRCPS, FFDRCSI, FDSRCSE, FRCPath, FMedSci, FHEA, FUCL, FSB, DSc, DChD, DMed(HC), Dr(HC) Emeritus Professor, University College London; Visiting Professor, Universities of Athens, BPP, Edinburgh, Granada, Helsinki and Plymouth

Crispian Scully, MD, MRCS, PhD, MDS, CBE, FDSRCS(Eng), FDSRCPS, FFDRCSI, FDSRCSE, FRCPath, FMedSci, FHEA, FUCL, FSB, DSc, DChD, DMed(HC), Dr(HC) is a member of the following medical societies: Academy of Medical Sciences, British Society for Oral Medicine, European Association for Oral Medicine, International Academy of Oral Oncology, International Association for Dental Research, International Association for Oral and Maxillofacial Pathology

Disclosure: Nothing to disclose.

Acknowledgements

David P Fivenson, MD Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.