$Unique_ID{BRK03724} $Pretitle{} $Title{Erythema Multiforme} $Subject{Erythema Multiforme Dermatostomatitis Erythema Multiforme Bullosum Erythema Polymorphe Febrile Mucocutaneous syndrome Herpes Iris} $Volume{} $Log{} Copyright (C) 1986, 1989, 1992 National Organization for Rare Disorders, Inc. 280: Erythema Multiforme ** IMPORTANT ** It is possible the main title of the article (Erythema Multiforme) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Dermatostomatitis Erythema Multiforme Bullosum Erythema Polymorphe Febrile Mucocutaneous syndrome Herpes Iris General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Erythema Multiforme is an inflammatory skin disorder characterized by symmetric red and blistery (bullous) lesions of the skin or mucous membranes of the hands, feet and eyelids. Symptoms Onset of Erythema Multiforme is usually sudden. Red spots (macules or papules), or ridges (wheals), and sometimes blisters, appear mainly on the back of hands, feet and face. Bleeding lesions on the lips and the mucous membranes in the mouth can also occur. The skin lesions are usually distributed on both sides of the body and they often appear as rings, with concentric circles having a grayish discoloration in the center. Itching can also occur. Systemic symptoms vary; malaise, pain in the joints (arthralgia), muscular stiffness and fever are frequent. Attacks usually last 2 to 4 weeks and may recur during the fall and spring for several years. Stevens-Johnson syndrome is a severe form of Erythema Multiforme. (For more information, choose "Stevens Johnson" as your search term in the Rare Diseases Database.) Causes In approximately 50% of cases of Erythema Multiforme, the cause is unknown. In children and young adults, infections caused by Herpes Simplex (probably the most commonly found etiologic agent), Coxsackie- and Echoviruses have been identified as the cause of Erythema Multiforme. The following may also result in this disorder: Mycoplasma pneumonia, Psittacosis, Histoplasmosis, and Vaccinia, Bacillus Calmette-Guerin, and Poliomyelitis vaccines. In other cases, x-ray therapy or drugs (e.g., penicillin, sulfonamides, and barbiturates) can sometimes cause Erythema Multiforme in susceptible people. While the precise mechanisms by which infectious agents or drugs cause the condition in some people is unknown, but it appears to be an unpredictable allergic reaction to these substances. In some cases Erythema Multiforme may occur during the latter half of pregnancy. In these cases the disorder usually disappears after delivery or if the pregnancy is terminated. Affected Population People of both sexes and all ages can be affected by Erythema Multiforme. Related Disorders Urticaria (hives) is easily recognized by the typical well-defined edematous ridges (wheals). This type of skin disorder does not include blisters. Erythema Nodosum is an inflammatory disease of the skin and subcutaneous tissue characterized by tender red nodules, predominantly appearing on the shins but occasionally involving the arms or other areas. Bullous Pemphigoid is a chronic benign blistery (bullous) skin eruption seen chiefly in the elderly. Dermatitis Herpetiformis (Duhring Disease) is a chronic skin eruption characterized by clusters of intensely itchy blisters, elevated spots (papules) and urticaria-like lesions. Pemphigus is an uncommon serious hereditary skin disorder characterized by blisters (bullae) in the top layer (epidermis) of apparently healthy skin and mucous membranes. (For more information on the above disorders, choose "urticaria," "pemphigoid," "Duhring," and "pemphigus" as your search terms in the Rare Disease Database.) Therapies: Standard When a cause for Erythema Multiforme can be found, it should be treated, eliminated, or avoided (e.g. drugs or other substances to which the patient is allergic). Local treatment depends on the type of lesion. Simple Erythema often requires no treatment and will remit with time. Blisters and erosive lesions can be treated with intermittent tap-water compresses. Infections of the lips and mouth with Erythema Multiforme may require special care. Oral hygiene is beneficial. A mouthwash of sodium bicarbonate solution in warm water is soothing and cleansing. Rinsing after each meal with elixir of dexamethasone can relieve discomfort and promote healing of nonviral oral lesions. Systemic corticosteroids have often been used in severe Erythema Multiforme, sometimes with apparent benefit. Other patients, especially those with severe mouth and throat lesions, can more readily contract serious respiratory infections if treated with systemic corticosteroids. Intensive systemic antibiotics, fluids, and electrolytes may be lifesaving in patients with extensive mucous membrane lesions. Therapies: Investigational The orphan drug thalidomide is being tested as a treatment for Erythema Multiforme. This drug should not be taken by pregnant women because it can cause severe birth defects. Physicians wishing to test thalidomide as a treatment for this disorder may contact: Pediatric Pharmaceutical 379 Thornall St. Edison, NJ 08837 Thalidomide is available in England under special license from Penn Pharmaceuticals of Tredegar, South Wales. This disease entry is based upon medical information available through January 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Erythema Multiforme, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References ERYTHEMA MULTIFORME: W. Stewart, et. al., eds; In: Dermatology: Diagnosis and Treatment of Cutaneous Disorders, 3rd edition; Mosby, 1974.