Cheilitis Granulomatosa Workup

Author: Crispian Scully, MD, PhD, MDS, CBE, MRCS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FRCPath, FMedSci, FHEA, FUCL, DSc, DChD, DMed(HC), Dr(HC), ; Chief Editor: Dirk M Elston, MD more...

Updated: Apr 20, 2012

What would you like to print?

Approach Considerations
Histologic Findings
Show All

Approach Considerations

Lab studies

Serum angiotensin-converting enzyme test may be performed to help exclude sarcoidosis. Patch tests may be used to help exclude reactions to metals, food additives, or other oral antigens^[17]; some cases of granulomatous cheilitis may be associated with such sensitivities. If found, avoidance of the implicated allergen is recommended.

Imaging studies

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 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.^[18]Small granulomas occur in the lymphatic walls in some cases. Similar changes may be present in cervical lymph nodes.

Proceed to Treatment & Management

Contributor Information and Disclosures

Author

Crispian Scully, MD, PhD, MDS, CBE, MRCS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FRCPath, FMedSci, FHEA, FUCL, DSc, DChD, DMed(HC), Dr(HC), Professor of Oral Medicine, Bristol University; Co-Director of World Health Organization Collaborating Centre for Oral Health-General Health; Emeritus Professor of Oral Medicine and Special Care Dentistry, University College London; Professor, Oral Medicine, Pathology, and Microbiology, University of London; Visiting Professor at Universities of Athens,Edinburgh, Granada, Helsinki and Plymouth

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

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

References

Ficarra G, Cicchi P, Amorosi A, Piluso S. Oral Crohn's disease and pyostomatitis vegetans. An unusual association. Oral Surg Oral Med Oral Pathol. Feb 1993;75(2):220-4. [Medline].
Khouri JM, Bohane TD, Day AS. Is orofacial granulomatosis in children a feature of Crohn's disease?. Acta Paediatr. Apr 2005;94(4):501-4. [Medline].
Narbutt P, Dziki A. Re: Melkersson-Rosenthal syndrome as an early manifestation of Crohn's disease. Colorectal Dis. Jul 2005;7(4):420-1. [Medline].
Scully C, Cochran KM, Russell RI, Ferguson MM, Ghouri MA, Lee FD, et al. Crohn's disease of the mouth: an indicator of intestinal involvement. Gut. Mar 1982;23(3):198-201. [Medline].
Williams AJ, Wray D, Ferguson A. The clinical entity of orofacial Crohn's disease. Q J Med. May 1991;79(289):451-8. [Medline].
Blinder D, Yahatom R, Taicher S. Oral manifestations of sarcoidosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Apr 1997;83(4):458-61. [Medline].
McCartan BE, Healy CM, McCreary CE, Flint SR, Rogers S, Toner ME. Characteristics of patients with orofacial granulomatosis. Oral Dis. Oct 2011;17(7):696-704. [Medline].
Ketabchi S, Massi D, Ficarra G, et al. Expression of protease-activated receptor-1 and -2 in orofacial granulomatosis. Oral Dis. Jul 2007;13(4):419-25. [Medline].
Tilakaratne WM, Freysdottir J, Fortune F. Orofacial granulomatosis: review on aetiology and pathogenesis. J Oral Pathol Med. Apr 2008;37(4):191-5. [Medline].
Saalman R, Sundell S, Kullberg-Lindh C, Lövsund-Johannesson E, Jontell M. Long-standing oral mucosal lesions in solid organ-transplanted children-a novel clinical entity. Transplantation. 2010;89:606.
McKenna KE, Walsh MY, Burrows D. The Melkersson-Rosenthal syndrome and food additive hypersensitivity. Br J Dermatol. Dec 1994;131(6):921-2. [Medline].
Morales C, Penarrocha M, Bagan JV, Burches E, Pelaez A. Immunological study of Melkersson-Rosenthal syndrome. Lack of response to food additive challenge. Clin Exp Allergy. Mar 1995;25(3):260-4. [Medline].
Wong GA, Shear NH. Melkersson-Rosenthal syndrome associated with allergic contact dermatitis from octyl and dodecyl gallates. Contact Dermatitis. Nov 2003;49(5):266-7. [Medline].
White A, Nunes C, Escudier M, et al. Improvement in orofacial granulomatosis on a cinnamon- and benzoate-free diet. Inflamm Bowel Dis. Jun 2006;12(6):508-14. [Medline].
Khandpur S, Malhotra AK, Khanna N. Melkersson-Rosenthal syndrome with diffuse facial swelling and multiple cranial nerve palsies. J Dermatol. Jun 2006;33(6):411-4. [Medline].
Saito T, Hida C, Tsunoda I, Tsukamoto T, Yamamoto T. Melkersson-Rosenthal syndrome: distal facial nerve branch palsies, masseter myopathy and corticosteroid treatment. Fukushima J Med Sci. Jun 1994;40(1):39-44. [Medline].
Fitzpatrick L, Healy CM, McCartan BE, Flint SR, McCreary CE, Rogers S. Patch testing for food-associated allergies in orofacial granulomatosis. J Oral Pathol Med. Jan 2011;40(1):10-3. [Medline].
[Guideline] Grave B, McCullough M, Wiesenfeld D. Orofacial granulomatosis--a 20-year review. Oral Diseases. 2009;15:46-51.
Campbell HE, Escudier MP, Patel P, Challacombe SJ, Sanderson JD, Lomer MC. Review article: cinnamon- and benzoate-free diet as a primary treatment for orofacial granulomatosis. Aliment Pharmacol Ther. Oct 2011;34(7):687-701. [Medline].
Williams PM, Greenberg MS. Management of cheilitis granulomatosa. Oral Surg Oral Med Oral Pathol. Oct 1991;72(4):436-9. [Medline].
Kruse-Losler B, Presser D, Metze D, Joos U. Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Arch Dermatol. Sep 2005;141(9):1085-91. [Medline].
Kano Y, Shiohara T, Yagita A, Nagashima M. Treatment of recalcitrant cheilitis granulomatosa with metronidazole. J Am Acad Dermatol. Oct 1992;27(4):629-30. [Medline].
Fdez-Freire LR, Serrano Gotarredona A, Bernabeu Wittel J, Pulpillo Ruiz A, Cabrera R, Navarrete Ortega M, et al. Clofazimine as elective treatment for granulomatous cheilitis. J Drugs Dermatol. May-Jun 2005;4(3):374-7. [Medline].
Tonkovic-Capin V, Galbraith SS, Rogers RS 3rd, Binion DG, Yancey KB. Cutaneous Crohn's disease mimicking Melkersson-Rosenthal syndrome: treatment with methotrexate. J Eur Acad Dermatol Venereol. Apr 2006;20(4):449-52. [Medline].
Barry O, Barry J, Langan S, Murphy M, Fitzgibbon J, Lyons JF. Treatment of granulomatous cheilitis with infliximab. Arch Dermatol. Sep 2005;141(9):1080-2. [Medline].
Sobjanek M, Wlodarkiewicz A, Zelazny I, Nowicki R, Michajlowski I, Sokolowska-Wojdylo M. Successful treatment of Melkersson-Rosenthal syndrome with dapsone and triamcinolone injections. J Eur Acad Dermatol Venereol. Aug 2008;22(8):1028-9; author reply 1027-8. [Medline].
Hazey MA, Van Norman AJ, Armistead DL. Melkersson-Rosenthal Syndrome with migraine-like headaches treated with minocycline: a case report and review of the literature. W V Med J. Jan-Feb 2009;105(1):15-7. [Medline].
Ishiguro E, Hatamochi A, Hamasaki Y, Ishikawa S, Yamazaki S. Successful treatment of granulomatous cheilitis with roxithromycin. J Dermatol. Sep 2008;35(9):598-600. [Medline].

Labial swelling and angular cheilitis.

Orofacial granulomatosis in a patient with Crohn disease.

View Table List

Read more about Cheilitis Granulomatosa on Medscape

About Medscape Reference

[ CLOSE WINDOW ]

About Medscape Reference

Medscape Reference is the most authoritative and accessible point-of-care medical reference for physicians and health care professionals. All content is available free of charge, both online and via mobile devices.

Medscape Reference articles represent the expertise and practical knowledge of top physicians and pharmacists from leading academic medical centers in the US and worldwide. In addition, our rigorous literature survey process allows us to rapidly integrate new practice-changing information into the relevant topics by systematically reviewing the major medical and pharmacy journals, news announcements, and important practice guidelines.

Medscape Reference provides comprehensive coverage across more than 30 medical specialties and is composed of the following areas:

Diseases and Conditions

More than 6,300 evidence-based and physician-reviewed disease and condition articles are organized to rapidly and comprehensively answer clinical questions, as well as to provide in-depth information in support of diagnosis, treatment, and other clinical decision-making. Topics are richly illustrated with more than 30,000 clinical photos and radiographic images.

Procedures

More than 650 clinical procedure articles provide clear, step-by-step instructions, and include instructional videos and images to allow clinicians to master the newest techniques or to improve their skills in procedures they have previously performed.

Drugs

More than 2,100 monographs, including prescription and over-the-counter drugs, plus over 5,000 corresponding brand-name drugs, herbals, and supplements, are provided. Drug images and pricing are also included. In addition, a Drug Interaction Checker provides rapid access to tens of thousands of interactions between brand and generic drugs, over-the-counter drugs, and herbals and supplements.

Anatomy

More than 70 anatomy articles feature clinical images and diagrams of the major human body systems and organs. The articles assist with the understanding of the anatomy involved in treating specific conditions and performing procedures. They can also facilitate physician-patient discussions.

Special Features
Best Evidence

This email newsletter features current and clinically relevant journal citations that are selected via our rigorous literature survey review process. These same citations are incorporated into the relevant Medscape Reference articles. This newsletter serves as a quick and easily accessible way to stay up to date with clinically relevant journal articles that impact clinical practice.

Case Studies

These challenging and classic case presentations provide a quick way for practicing physicians to sharpen their diagnostic and patient-management skills. Each case starts with how the patient presented in the clinic and then using a question and answer format, progresses through diagnosis and on to treatment and includes follow up information about outcome for the patient.

Image collections

Image collections are a visually-engaging presentation of both common and uncommon diseases, case presentations, and current controversies in medicine. They are presented in an image rich, slideshow format and are designed to challenge and expand physicians' knowledge of clinically important topics.

Medscape is the leading online destination for healthcare professionals for clinical information. In addition to clinical reference tools, Medscape also offers:

Medscape News Learn more
Medscape Education Learn more