Cheilitis Granulomatosa Clinical Presentation

  • 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
 

History

Cheilitis granulomatosa is episodic with nontender swelling and enlargement of 1 or both lips. Occasionally, similar swellings involve other areas, including the periocular region.

A fissured or plicated tongue is seen in 20-40% of patients. Its presence from birth (in some patients) may indicate a genetic susceptibility. Patients may lose the sense of taste and have decreased salivary gland secretion.

The first episode of edema typically subsides completely in hours or days. After recurrent attacks, swelling may persist and slowly increase in degree, eventually becoming permanent. Recurrences can range from days to years. Attacks sometimes are accompanied by fever and mild constitutional symptoms (eg, headache, visual disturbance). Melkersson-Rosenthal syndrome involves the association with facial nerve palsy.[15, 16]

Facial palsy of the lower motor-neuron type occurs in about 30% of patients with granulomatous cheilitis. Facial palsy may precede facial swelling by months or years, but it more commonly develops later. Facial palsy is intermittent at first, but it may become permanent. It can be unilateral or bilateral, partial or complete. Other cranial nerves (eg, olfactory, auditory, glossopharyngeal, hypoglossal) are occasionally affected.

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Physical Examination

The earliest manifestation of granulomatous cheilitis is sudden diffuse or occasionally nodular swellings of the lip or the face involving (in decreasing order of frequency) the upper lip, the lower lip, and 1 or both cheeks. The forehead, the eyelids, or 1 side of the scalp may be involved (less common). As previously mentioned, a fissured or plicated tongue is seen in 20-40% of patients.

The swelling may feel soft, firm, or nodular on palpation. Once chronicity is established, the enlarged lip appears cracked and fissured, with reddish brown discoloration and scaling. The fissured lip becomes painful and eventually acquires the consistency of firm rubber. Swelling may regress very slowly after some years. Regional lymph nodes are enlarged (usually minimally) in 50% of patients.

Facial palsy of the lower motor-neuron type occurs in up to 30% of patients. It can be unilateral or bilateral, partial or complete. Other cranial nerves (eg, olfactory, auditory, glossopharyngeal, hypoglossal) are occasionally affected.

Central nervous system involvement has been reported, but the significance of resulting symptoms is easily overlooked because they are very variable (sometimes simulating multiple sclerosis but often with a poorly defined association of psychiatric and neurologic features). Autonomic disturbances may occur.

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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

  1. 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].

  2. 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].

  3. 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].

  4. 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].

  5. Williams AJ, Wray D, Ferguson A. The clinical entity of orofacial Crohn's disease. Q J Med. May 1991;79(289):451-8. [Medline].

  6. 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].

  7. 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].

  8. 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].

  9. Tilakaratne WM, Freysdottir J, Fortune F. Orofacial granulomatosis: review on aetiology and pathogenesis. J Oral Pathol Med. Apr 2008;37(4):191-5. [Medline].

  10. 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.

  11. McKenna KE, Walsh MY, Burrows D. The Melkersson-Rosenthal syndrome and food additive hypersensitivity. Br J Dermatol. Dec 1994;131(6):921-2. [Medline].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. 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].

  18. [Guideline] Grave B, McCullough M, Wiesenfeld D. Orofacial granulomatosis--a 20-year review. Oral Diseases. 2009;15:46-51.

  19. 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].

  20. Williams PM, Greenberg MS. Management of cheilitis granulomatosa. Oral Surg Oral Med Oral Pathol. Oct 1991;72(4):436-9. [Medline].

  21. 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].

  22. 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].

  23. 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].

  24. 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].

  25. 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].

  26. 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].

  27. 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].

  28. 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].

 
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Orofacial granulomatosis in a patient with Crohn disease.
 
 
 
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