Erythema multiforme

Erythema multiforme on the leg

Erythema multiforme on the hand

Exfoliation following erythroderma

Erythema multiforme

Definition:

Erythema multiforme is a skin disorder resulting from an allergic reaction.

Alternative Names:

Lyell's syndrome; Stevens-Johnson syndrome; Toxic epidermal necrolysis

Causes, incidence, and risk factors:

Erythema multiforme is a type of hypersensitivity (allergic) reaction that occurs in response to medications, infections, or illness. Medications associated with erythema multiforme include sulfonamides, penicillins, barbiturates, and phenytoin. Associated infections include herpes simplex and mycoplasma infections.

The exact cause is unknown. The disorder is believed to involve damage to the blood vessels of the skin with subsequent damage to skin tissues. Approximately 90% of erythema multiforme cases are associated with herpes simplex or Mycoplasma infections. The disorder occurs primarily in children and young adults.

Erythema multiforme may present with a classic skin lesion with or without systemic (whole body) symptoms. In Stevens-Johnson syndrome, the systemic symptoms are severe and the lesions are extensive, involving multiple body areas (especially the mucous membranes). Toxic epidermal necrolysis (TEN syndrome, or Lyell's syndrome) involves multiple large blisters (bullae) that coalesce, followed by sloughing of all or most of the skin and mucous membranes.

Symptoms:

Skin lesion
- Multiple
- With sudden onset, may recur
- May spread
- May appear as nodule, papule, or macule
- Central lesion surrounded by concentric rings of pallor and redness ("target", "iris", "bull's eye" shape)
- May have vesicles and bullae (blisters of various sizes)
- Located on the legs, arms, palms, hands, or feet
- May involve the face or lips
- Trunk is usually not involved
- Usually symmetrical
Itching of the skin may be present
Fever
General ill feeling (malaise)
Joint aches

Additional symptoms that may be associated with this disease:

Signs and tests:

Positive Nikolsky's sign

The diagnosis is primarily based on the classic skin lesion appearance and typical symmetrical distribution, especially if there is a history of risk factors or associated diseases.

A skin lesion biopsy and microscopic examination may be helpful to differentiate erythema multiforme from other disorders. Erythema multiforme may show keratinocyte necrosis (tissue death) and other changes. Microscopic examination of the tissue may also show deposits of immune complexes (antibody deposits).

Treatment:

Treatment goals include control of the underlying causes or illnesses, treatment of the symptoms, and prevention of infection. Suspected medications should be discontinued.

Treatment of mild symptoms may include:

Moist compresses applied to skin lesions.
Medications such as antihistamines to control itching.
Over-the-counter medications (such as acetaminophen) to reduce fever and discomfort.
Topical anesthetics (especially for mouth lesions) to ease discomfort that interferes with eating and drinking.

Treatment of severe symptoms may include:

Hospitalization and treatment in an intensive care or burn care unit for severe cases, Stevens-Johnson syndrome, and toxic epidermal necrolysis
Systemic corticosteroids to control inflammation
Intravenous immunoglobulins (IVIG) to stop the process
Antibiotics to control secondary skin infections

Good hygiene and isolation from others may be required to prevent secondary infections.

Extensive skin involvement may cause the loss of large quantities of body fluids, causing shock in addition to the risk of infection. Intensive care with support of body systems may be required.

Skin grafting may be helpful in cases in which large areas of the body are affected.

Expectations (prognosis):

Mild forms of erythema multiforme usually resolve without difficulty in 2 to 6 weeks, but they may recur. More severe forms may be difficult to treat. Stevens-Johnson syndrome and toxic epidermal necrolysis are associated with high death rates.

Complications:

Permanent skin damage and scarring
Occasionally, lesions on internal organs causing:
- Pneumonitis (lung inflammation)
- Myocarditis (heart inflammation)
- Nephritis (kidney inflammation)
- Hepatitis (liver inflammation)
Secondary skin infection (cellulitis)
Systemic infection, sepsis
Loss of body fluids, shock

Calling your health care provider:

Go to the emergency room or call the local emergency number (such as 911) if symptoms indicate erythema multiforme. Involvement of a large area of the body is an emergency situation.

Review Date: 7/28/2001
Reviewed By: Michael Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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