Erythema Multiforme

Erythema Multiforme: Excerpt from The 5-Minute Pediatric Consult

Albert C. Yan, MD

Erythema Multiforme - BASICS

Erythema Multiforme - description

An acute self-limited cutaneous eruption with many different or multiform lesions:

• Characterized classically as a target or iris lesion but may appear as erythematous macules, papules, vesicles, and bullae with mucosal involvement
• There are many triggers of erythema multiforme (EM), which is thought to encompass a spectrum of disease from relatively mild disease (EM minor) to severe forms with more than 1 mucosal surface involved (EM major or Stevens-Johnson syndrome).
• Some authors include toxic epidermal necrolysis (TEN) as the most severe form of EM, characterized by widespread erythema, bullae, and sloughing of large sheets of skin, with significant morbidity and mortality.
• Some debate exists as to whether classic EM minor may represent an entity altogether separate from Stevens-Johnson and TEN.

Erythema Multiforme - epidemiology

• Believed by some to occur more frequently in spring and summer, with the more severe form of EM major occurring in winter
• Occurs predominantly in young adults

Erythema Multiforme - incidence

Male = Female (some studies suggest a slightly higher incidence of EM minor in women)

Erythema Multiforme - prevalence

Seen in ~1% of all dermatology patients

Erythema Multiforme - risk factors

Erythema Multiforme - genetics

Although simultaneous cases in family members have been reported, the disease is not genetic.

Erythema Multiforme - etiology

• The major causes of EM, which is thought to be an immune-mediated reaction, include drugs such as sulfa, penicillin, and phenytoin and infections such as herpes simplex virus and Mycoplasma.
• There are a host of other etiologic factors, including exposure to various chemicals and tumors. The eruption usually occurs 1–2 weeks after the initial exposure.
• Often the causative factor is not identified.
• Recurrent EM is generally secondary to herpes simplex virus.

Erythema Multiforme - DIAGNOSIS

Erythema Multiforme - signs & symptoms

Erythema Multiforme - history

Cutaneous findings are sometimes preceded by a prodrome with fever and malaise. A careful drug and exposure history, as well as any signs or symptoms of infection or herpetic lesions, may reveal the cause. Inquire in detail about the patient’s drug history, OTC preparations, and signs or symptoms of infection or herpetic lesions.

Erythema Multiforme - physical exam

• EM classically appears as target lesions characterized by a dark, dusky center surrounded by a pale zone and then a zone of erythema. The lesions are typically acrally distributed.
• Lesions occur in many forms and may appear as red macules, papules, urticarial lesions, or vesicles and bullae.
• Oral involvement is typically seen. Mucosal involvement with superficial denudation may also occur in the eyes, nasopharyngeal mucosal, or anogenital region.

Erythema Multiforme - tests

There are no diagnostic laboratory tests; however, biopsy is often helpful, and other tests may help identify a cause.

Erythema Multiforme - lab

• A WBC count with differential, looking for eosinophilia, may help identify a drug as causative.
• Direct fluorescent antibody testing, polymerase chain reaction, and cultures to evaluate for herpes or chest radiographic studies to evaluate for pneumonia or infectious cause of EM
• Cold agglutinins, serology, and polymerase chain reaction associated with Mycoplasma
• Antistreptolysin-O titers and leukocytosis may identify a particular infectious cause.
• Erythrocyte sedimentation rate may be elevated, but is nonspecific.

Erythema Multiforme - diag proced-surgery

Biopsy of lesion

Erythema Multiforme - pathological findings

Vary according to the lesion examined:

• Biopsy reveals necrosis of keratinocytes to varying degrees, depending on the clinical lesion biopsied
• Moderate to severe papillary dermal edema with mild to moderate perivascular dermal infiltrate composed predominantly of mononuclear cells and also some eosinophils (particularly if drug related)
• Subepidermal blistering may be seen
• Extravasated blood cells are found, but there is no evidence of vasculitis
• Hydropic degeneration of the basement membrane also may be seen, as may epidermal spongiosis

Erythema Multiforme - differencial diagnosis

Classic presentation with targetoid lesions and mucosal involvement is generally not a diagnostic challenge; however, given the many forms of presentation, the diagnosis of EM may be difficult. The differential diagnosis may be extensive depending on the presentation and includes:

• Viral exanthem
• Bullous impetigo
• Staphylococcal scalded-skin syndrome
• Bullous pemphigoid
• Urticaria
• Urticarial vasculitis
• Systemic lupus erythematosus
• Serum sickness
• Pemphigus vulgaris
• Secondary syphilis
• Chickenpox
• Rocky Mountain spotted fever
• Acute neutrophilic dermatosis
• Lyme disease
• Fixed drug eruption

Erythema Multiforme - TREATMENT

Erythema Multiforme - initial stabilization

• Mild EM: Resolves spontaneously without scarring and require only supportive therapy, including antihistamine or topical steroid for pruritus associated with the lesions
• EM major may be life threatening and may require hospitalization.
• TEN: High associated mortality is often secondary to infection; care is ideally at a burn center, with careful attention to infection and to fluids and electrolytes.

Erythema Multiforme - general measures

• Mild forms:
  • Oral lesions are often painful, and oral preparations to swish and spit, made of diphenhydramine or viscous lidocaine, may provide relief.
  • Treatment of the underlying process is helpful (e.g., acyclovir for herpes simplex virus–associated cases).
• EM major:
  • Supportive care, ophthalmology consultation, monitoring of fluid and electrolyte balance, and vigilant observation for infection are necessary.
  • Antibiotics, analgesics, and local care including compresses with acetic acid soaks or saline will help decrease the incidence of infection.
  • The use of systemic steroids is controversial, but when helpful, they are given early in the course of disease for ~2 weeks when there is no contraindication, such as infection.
• TEN:
  • Antibiotics, local compresses with acetic acid, or saline may help prevent superinfection.
  • The use of systemic steroids is controversial and is most effective when started early. The benefits and risks, including infection, must be weighed.
  • Some recent reports demonstrated that IV immunoglobulin given over 3–5 days provided rapid improvement with little to no adverse effects in adult patients.

Erythema Multiforme - FOLLOW UP

Erythema Multiforme - prognosis

• Mild forms of EM are acute and self-limited, with lesions resolving in 2–4 weeks with postinflammatory hyperpigmentation or hypopigmentation.
• Sequelae owing to mucosal scarring may occur.
• Severe forms of EM major or TEN have associated morbidity and mortality.
• When recurrent, EM is often associated with herpes simplex virus.

Erythema Multiforme - complications

• EM minor is generally self-limited, with rare complications.
• In EM major, mucosal involvement may lead to stricture formation of the urethra, trachea, and esophagus, as well as conjunctivitis, corneal erosions, and rarely, blindness.
• Pneumonitis, nephritis, hepatitis, and infection are other reported complications.
• In TEN, mortality and morbidity are high, with death occurring from sepsis.

Erythema Multiforme - bibliography

1. Ayangco L, Rogers RS 3rd. Oral manifestations of erythema multiforme. Dermatol Clin. 2003;21:195–205.
Bondi EE, Jegasothy BV, Lazarus GS. Dermatology Diagnosis and Therapy. Norwalk, CT: Appleton & Lange; 1991.
2. Duvic M. Erythema multiforme. Dermatol Clin. 1983;1:493–496.
3. Huff JC, Weston WL, Tonnessen MG. Erythema multiforme: A critical review of characteristics, diagnostic criteria and causes. J Am Acad Dermatol. 1983;8:763–775.
Lever WF, Schaumberg-Lever G. Histopathology of the Skin. 7th ed. Philadelphia: Lippincott; 1990.
4. Metry DW, Jung P, Levy ML. Use of intravenous immunoglobulin in children with Stevens-Johnson syndrome and toxic epidermal necrolysis: seven cases and review of the literature. Pediatrics. 2003;112(6 pt 1):1430–1436.
5. Weston WL, Badgett JT. Urticaria. Pediatr Rev. 1998;19:240–244.

Erythema Multiforme - CODES

Erythema Multiforme - icd9

695.1 Erythema multiforme

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Stevens-Johnson Syndrome

• Back to disease: Stevens-Johnson Syndrome: Introduction
• Next Book Extract About Stevens-Johnson Syndrome: Stevens Johnson Syndrome (The 5-Minute Pediatric Consult)
• More Book Extracts: All Online Books for Stevens-Johnson Syndrome

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

 » Next page: Stevens Johnson Syndrome (The 5-Minute Pediatric Consult)

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