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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.