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- $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.
-
-