Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome) 

  • Author: Crispian Scully, MD, PhD, MDS, CBE, MDS, MRCS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FRCPath, FMedSci, FHEA, FUCL, DSc, DChD, DMed(HC), Dr(HC) ; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Feb 15, 2011
 

Background

Granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation. Miescher cheilitis is the term used when the granulomatous changes are confined to the lip. Miescher cheilitis is generally regarded as a monosymptomatic form of the Melkersson-Rosenthal syndrome, although the possibility remains that these may be 2 separate diseases. Melkersson-Rosenthal syndrome is the term used when cheilitis occurs with facial palsy and plicated tongue. Note the image below.

Labial swelling and angular cheilitis. Labial swelling and angular cheilitis.

Melkersson-Rosenthal syndrome is occasionally a manifestation of Crohn disease[1, 2, 3, 4, 5] or orofacial granulomatosis (OFG).

Next

Pathophysiology

In granulomatous cheilitis, normal lip architecture is eventually altered by the presence of lymphoedema and noncaseating granulomas in the lamina propria. T H 1 immunocytes produce interleukin 12 and RANTES/MIP-1alpha and granulomas. Expression of protease-activated receptor 1 and 2 occurs in OFG. HLA typing may show HLA-A2 or HLA-A11.[6]

Previous
Next

Epidemiology

Frequency

International

The frequency is unknown; granulomatous cheilitis is rare.

Mortality/Morbidity

Morbidity related to granulomatous cheilitis depends on whether underlying organic disease, such as Crohn disease or sarcoidosis,[7] is present.

Race

No racial predilection is recognized for granulomatous cheilitis.

Sex

No sexual predilection is known for granulomatous cheilitis.

Age

Onset of granulomatous cheilitis usually occurs in young adult life.

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Crispian Scully, MD, PhD, MDS, CBE, MDS, MRCS, FDSRCS, FDSRCPS, FFDRCSI, FDSRCSE, FRCPath, FMedSci, FHEA, FUCL, DSc, DChD, DMed(HC), Dr(HC)  Professor of Oral Medicine, Bristol University; 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, Bristol, Edinburgh, Granada, and Helsinki

Crispian Scully, MD, PhD, MDS, CBE, MDS, 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 Royal Society of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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.

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.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

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. 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].

  7. 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].

  8. 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].

  9. 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].

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

  11. 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.

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

  13. 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].

  14. 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].

  15. 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].

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

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

  18. 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].

  19. 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].

  20. 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].

  21. 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].

  22. 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].

  23. 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].

  24. 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].

  25. 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].

 
Previous
Next
 
Labial swelling and angular cheilitis.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.