National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Erythema Multiforme is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- Dermatostomatitis, Erythema Multiforme Type
- Erythema Multiforme Bullosum
- Erythema Polymorphe, Erythema Multiforme Type
- Febrile Mucocutaneous Syndrome
- Herpes Iris, Erythema Multiforme Type
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
- Erythema Nodosum
- Bullous Pemphigoid
- Dermatitis Herpetiformis
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).
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.
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.
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.
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.)
Usually, the diagnosis can be made on the basis of the size, shape, color and distribution of the target lesions.
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.
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FROM THE INTERNET
Kantor J. Erythema multiforme. Medical Encyclopedia. MedlinePlus. Update date: 10/29/2004. 3pp.
Pruksachatkunakorn C. Schachner L. Erythema Multiforme. emedicine. Last Updated: October 7, 2004. 12pp.
Small Pox Vaccination and Adverse Events Training Module. CDC. May 2003, 9pp.
Weston WL. Erythema Multiforme. Children's Hospital and Regional Medical Center. nd. 3pp.
NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases
One AMS Circle
Bethesda, MD 20892-3675
Stevens Johnson Syndrome Foundation and Support Group
PO Box 350333
Westminster, CO 80035-0333
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
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