Melkersson Rosenthal Syndrome

Melkersson Rosenthal Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Melkersson Rosenthal Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • MROS
  • Melkersson Syndrome
  • MRS

Disorder Subdivisions

  • None

General Discussion

Melkersson Rosenthal syndrome is a rare neurological disorder characterized by recurrent, long lasting swelling of the face, particularly one or both lips (granulomatous cheilitis), facial muscle weakness (palsy) and a fissured tongue. Some affected individuals may have all three of these features and others may have only one or two.

Symptoms

The first symptom of MRS is usually swelling of the upper lip, lower lip, one or both cheeks, eyelids, or rarely, one side of the scalp. The first episode may resolve in hours or days, but swelling may be more severe and last longer in subsequent episodes and can become permanent. The enlarged lips may appear cracked and discolored and can be painful. Fever, headache and visual disturbances sometimes occur with an episode. A fissured tongue is seen in 20-40% of those affected and may be present since birth. Salivary gland secretion may be reduced and the sense of taste may be diminished. Facial palsy occurs in about 30% of those affected. It usually occurs after episodes of lip swelling have already occurred but is sometimes the first symptom. The facial palsy can be on one side or both, resolves initially, but can become permanent.

Causes

MRS is thought to be caused by genetic factors in some cases because families have been described in which multiple members are affected. MRS is sometimes a symptom of another condition such as Crohn's disease or sarcoidosis. Dietary and other allergens may also be involved.

Affected Populations

Melkersson Rosenthal syndrome usually begins in young adulthood and affects slightly more females than males. Approximately 300 cases have been reported but the condition may be under-diagnosed because facial palsy is not always present.

Standard Therapies

Diagnosis

MRS is diagnosed by physical findings and history. A biopsy of the lips may be necessary to confirm the diagnosis in some cases. Specialized laboratory studies and procedures may be recommended to rule out Crohn's disease and sarcoidosis.



Treatment

Most signs and symptoms of MRS resolve without treatment but episodes may occur more often and last longer if the condition is not treated. Treatment for MRS may include corticosteroid injections, nonsteroidal anti-inflammatory agents and antibiotics. Surgery and/or radiation are sometimes recommended to reduce abnormally swollen lips.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Dutt SN and Irving RM. Melkersson-Rosenthal Syndrome. In: The NORD Guide to Rare Disorders, Philadelphia: Lippincott, Williams and Wilkins, 2003:557.



JOURNAL ARTICLES

Dutt SN, Mizra S, Irving RM, et al. Total decompression of facial nerve for Melkerrson-Rosenthal syndrome. J Laryngol Otol 2000;114:870-873.



Kovich OI and Cohen DE. Granulomatous cheilitis. Dermatology Online Journal 2004;10(3):10.



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



Scully, C. Cheilitis Granulomatosa (Miescher-Melkersson-Rosenthal Syndrome. EMedicine. Last Updated 2/2/05.



Shapiro M, Peters S, Spinelli HM: Melkersson-Rosenthal syndrome in the periocular area: a review of the literature and case report. Ann Plast Surg 2003; 50(6): 644-8.



Wong GA, Shear NH: Melkersson-Rosenthal syndrome associated with allergic contact dermatitis from octyl and dodecyl gallates. Contact Dermatitis 2003; 49(5): 266-7.



Zimmer WM, Rogers RS 3rd, Reeve CM, Sheridan PJ: Orofacial manifestations of Melkersson-Rosenthal syndrome. A study of 42 patients and review of 220 cases from the literature. Oral Surg Oral Med Oral Pathol 1992; 74(5): 610-9.



FROM THE INTERNET

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore, MD: The Johns Hopkins University; Entry No. 155990; Last Update: 3/17/04.

Resources

March of Dimes Birth Defects Foundation

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White Plains, NY 10605

Tel: (914)997-4488

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Email: Askus@marchofdimes.com

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American Autoimmune Related Diseases Association, Inc.

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Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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