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.
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.
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.
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.
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.
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
For information about clinical trials sponsored by private sources, contact:
Dutt SN and Irving RM. Melkersson-Rosenthal Syndrome. In: The NORD Guide to Rare Disorders, Philadelphia: Lippincott, Williams and Wilkins, 2003:557.
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.
The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.
National Organization for Rare Disorders (NORD)
55 Kenosia Ave., Danbury CT 06810 • (203)744-0100