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Erythema Multiforme

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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Synonyms of Erythema Multiforme

Disorder Subdivisions

General Discussion

Erythema multiforme (EM) is the name applied to a group of hypersensitivity disorders, affecting mostly children and young adults, and characterized by symmetric red, patchy lesions, primarily on the arms and legs. The cause is unknown, but EM frequently occurs in association with herpes simplex virus, suggesting an immunologic process initiated by the virus. In half of the cases, the triggering agents appear to be medications, including anticonvulsants, sulfonamides, nonsteroidal anti-inflammatory drugs, and other antibiotics. In addition, some cases appear to be associated with infectious organisms such as Mycoplasma pneumoniae and many viral agents.

Erythema multiforme is the mildest of three skin disorders that are often discussed in relation to each other. It is generally the mildest of the three. More severe is Stevens-Johnson syndrome. The most severe of the three is toxic epidermal necrolysis (TEN).

Symptoms

Onset of erythema multiforme is usually sudden in an otherwise healthy individual. Red spots (macules or papules), or ridges (wheals), and sometimes blisters appear on the tops of the hands and forearms. Other areas of involvement may include the face, neck, palms, soles of feet, legs, and trunk. The lesions continue to erupt for two or three days. Some spots, especially on the hands and forearms, may evolve into concentric circles that resemble a target, with a grayish discoloration in the center. A crust may develop over the center. In about half of the cases, lesions may develop on the lips and the mucous membranes in the mouth. The skin lesions are usually distributed on both sides of the body. Itching can also occur.

Systemic symptoms vary, but malaise, pain in the joints (arthralgia), muscular stiffness and fever are frequent. Additional symptoms may include vision abnormalities; dry or bloodshot eyes; and eye pain, itching, or burning.

Attacks usually last two to four weeks, and may recur. Classic EM tends to recur two or three times a year for several years after its first appearance.

Causes

The cause of erythema multiforme is unknown, but it appears to be an allergic reaction that occurs in response to medications, infections, or illness. As noted above, it often appears in association with herpes simplex virus or with infectious organisms such as Mycoplasma pneumoniae.

In approximately half of the cases, it appears that the triggering agent is a medication. Drugs that have been associated with erythema multiforme include anticonvulsants, sulfonamides, nonsteroidal anti-inflammatory drugs, and other antibiotics.

Affected Populations

Erythema multiforme is a rare disorder that affects slightly more males than females. It may begin at any age, but is most common in children and young adults.

Related Disorders

Symptoms of the following disorders can be similar to those of Erythema Multiforme. Comparisons may be useful for a differential diagnosis:

Urticaria (hives) is easily recognized by the typical well-defined edematous ridges (wheals). This type of skin disorder does not include blisters. (For more information on this disorder, choose "Urticaria" as your search term in the Rare Disease Database.)

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. (For more information on this disorder, choose "Erythema Nodosum" as your search term in the Rare Disease Database.)

Bullous Pemphigoid is a chronic benign blistery (bullous) skin eruption seen chiefly in the elderly. (For more information on this disorder, choose "Bullous Pemphigoid" as your search term in the Rare Disease Database.)

Dermatitis Herpetiformis (Duhring Disease) is a chronic skin eruption characterized by clusters of intensely itchy blisters, elevated spots (papules) and urticaria-like lesions. (For more information on this disorder, choose "Duhring" as your search term in the Rare Disease Database.)

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 this disorder, "Pemphigus" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
Usually, the diagnosis can be made on the basis of the size, shape, color and distribution of the target lesions.

Treatment
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.

Most people with classic erythema multiforme can be treated as outpatients with therapy, such as antihistamines, addressing their symptoms. Sometimes, no treatment is required.

For blisters and erosive lesions, intermittent moist compresses may be helpful. Over-the-counter antihistamines usually take care of whatever itching may be present. Infections of the lips and mouth usually can be managed with topical anesthetics but may, in some cases, require special care.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Erythema Multiforme Resources

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at solivo@rarediseases.org.)

Other Organizations:

References

TEXTBOOKS
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:757-58, 824.

Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1200.

Champion RH, Burton JL, Ebling FJG, eds. Textbook of Dermatology. 5th ed. Blackwell Scientific Publications. London, UK; 1992:1834-37.

REVIEW ARTICLES
Williams PM, Conklin RJ. Erythema multiforme: a review and contrast from Stevens-Johnson syndrome/toxic epidermal necrolysis. Dent Clin North Am. 2005;49:67-76.

Sidbury R. What's new in pediatric dermatology: update for the pediatrician. Curr Opin Pediatr. 2004;16:410-14.

Ahmed I, Reichenberg J, Lucas A, et al. Erythema multiforme associated with phenytoin and cranial radiation therapy: a report of three patients and review of the literature. Int J Dermatol. 2004;43:67-73.

Bachot N, Roujeau JC. Intravenous immunoglobulins in the treatment of severe drug eruptions. Curr Opin Allergy Clin Immunol. 2003;3:269-74.

Conejo-Mir JS. del Canto S, Munoz MA, et al. Thalidomide as elective treatment in persistent erythema multiforme; report of two cases. J Drugs Dermatol. 2003;2:40-44.

Aurelian L, Ono F, Burnett J. herpes simplex virus (HSV)-associated erythema multiforme (HAEM): a viral disease with an autoimmune component. Dermatol Online J. 2003;9:1.

Witkowski JA, Parish LC. Cutaneous reactions to antibacterial agents. Skinmed. 2002;1:33-44.

FROM THE INTERNET
Kantor J. Erythema multiforme. Medical Encyclopedia. MedlinePlus. Update date: 10/29/2004. 3pp.
www.nlm.nih.gov/medlineplus/ency/article/000851.htm

Pruksachatkunakorn C. Schachner L. Erythema Multiforme. emedicine. Last Updated: October 7, 2004. 12pp.
www.emedicine.com/derm/topic137.htm

Small Pox Vaccination and Adverse Events Training Module. CDC. May 2003, 9pp.
www.bt.cdc.gov/training/smallpoxvaccine/reactions/ery-multi.html

Weston WL. Erythema Multiforme. Children's Hospital and Regional Medical Center. nd. 3pp.
www.pediatricweb.com/seattle/article.asp?ArticleID=802&ArticleType=9

Report last updated: 2008/04/25 00:00:00 GMT+0