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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.
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.
Bell's palsy is a nonprogressive neurological disorder of one of the facial nerves (7th cranial nerve). This disorder is characterized by the sudden onset of facial paralysis that may be preceded by a slight fever, pain behind the ear on the affected side, a stiff neck, and weakness and/or stiffness on one side of the face. Paralysis results from decreased blood supply (ischemia) and/or compression of the 7th cranial nerve. The exact cause of Bell's palsy is not known. Viral (e.g., herpes zoster virus) and immune disorders are frequently implicated as a cause for this disorder. There may also be an inherited tendency toward developing Bell's palsy. (For more information on this disorder, choose "Bell" as your search term in the Rare Disease Database).
Crohn's disease is an inflammatory bowel disease characterized by severe, chronic inflammation of the intestinal wall or any portion of the gastrointestinal tract. The lower portion of the small intestine (ileum) and the rectum are most commonly affected by this disorder. Symptoms may include watery diarrhea, abdominal pain, fever, and weight loss. The symptoms of Crohn's disease can be difficult to manage and proper diagnosis is often delayed. The exact cause of Crohn's disease is unknown. (For more information on this disorder, choose "Crohn" as your search term in the Rare Disease Database).
Sarcoidosis is characterized by the abnormal formation of inflammatory masses or nodules (granulomas) consisting of certain granular white blood cells (modified macrophages or epithelioid cells) in certain organs of the body. The granulomas that are formed are thought to alter the normal structure of and, potentially, the normal functions of the affected organs. Granuloma formation most commonly affects the lungs, however, the upper respiratory system, lymph nodes, skin, and/or eyes may be involved. Other organs may also be affected, including the liver, bone marrow, spleen, musculoskeletal system, heart, salivary glands, and/or nervous system (i.e., central or peripheral nervous system). For more information on this disorder, choose "sarcoidosis" as your search term in the Rare Disease Database).
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.
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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.
Report last updated: 2007/09/23 00:00:00 GMT+0