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