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Erythema multiforme
OVERVIEW: Erythema multiforme (EM) is an acute self limited hypersensitivity reaction involving the skin and sometimes the mucus membranes. Erythema multiforme minor- also called the erythema multiforme - Hebra, is a mild form appearing as a pleomorphic rash which includes target lesions, but not large vesicles or petechia, affecting the skin with or without involving one mucus membrane site. Erythema multiforme major is a more severe form, involving more than one mucus membrane site. It was formerly considered to be the same as Stevens-Johnson syndrome, but is now believed to be unrelated.
System(s) affected: Skin/exocrine
Genetics: Possibly associated with HLA-B15
Incidence/Prevalence in USA: Not known. In Germany the incidence of erythema multiforme major, Stevens Johnson syndrome and toxic epidermal necrolysis combined appears to be 1.89 per million persons per year.
Predominant age: Peak incidence in 20's and 30's; rare under 3 and over age 50
Predominant sex: Male > Female (3:2)
SIGNS AND SYMPTOMS: • The typical pleomorphic eruption is a mixture of macules of various sizes and target lesions. These consist of a central inflamed and superficially necrotic area, surrounded by a halo of less inflamed skin, enclosed within an outer erythematous rim. Purpuric lesions are uncommon, and vesicles may be related to antecedent herpes I infections. The rash occurs on the palms, soles, dorsum of the hands and extensor surface of the extremities and the face. It is often recurrent, following a viral infection.
• Involvement of the mucus membranes is quite common, which caused confusion with Stevens Johnson syndrome. In EM mucus membrane involvement consists of target lesions of the lips or herpetic lesions without extensive necrosis.
• Pruritus is usually absent
• The skin may feel normal, or there may be a mild burning sensation.
• If corneal ulceration occurs it is a serious complication
CAUSES: • Most cases appear to be due to a preceding infection. Drugs seem to be an infrequent cause.
• Viral infections - particularly herpes simplex; also Epstein-Barr, Coxsackie, echovirus, varicella, mumps and poliovirus
• Bacterial infections- including Brucellosis, diphtheria (etc.), borreliosis. Mycoplasma appears to more often precede Stevens-Johnson syndrome.
• Protozoan infections
• Fungal infection, including Trichophyton rubrum
• Collagen vascular diseases
• Malignancy
• Pregnancy
• Premenstrual hormone changes
• Consumption of beer
• Reiter's syndrome
• Sarcoidosis
• Vaccines- tetanus/diphtheria (Td), bacillus Calmette-Guerin (BCG), oral polio vaccine (OPV)
• Medications- the accepted list includes sulfonamides, penicillins, anticonvulsants, salicylates, but many of these were actually associated with Stevens Johnson syndrome when it was considered to be a type of erythema nodosum major
• Radiotherapy
RISK FACTORS: • Previous history of erythema multiforme
• Male sex
DIAGNOSIS DIFFERENTIAL DIAGNOSIS: • Stevens Johnson syndrome
• Urticaria
• Necrotizing vasculitis
• Drug eruptions
• Contact dermatitis
• Pityriasis rosea
• Secondary syphilis
• Ringworm
• Pemphigus vulgaris
• Pemphigoid
• Dermatitis herpetiformis
• Herpes gestationis
• Septicemia
• Serum sickness
• Viral exanthems
• Rocky Mountain spotted fever
• Collagen vascular diseases
• Mucocutaneous lymph node syndrome
• Meningococcemia
• Lichen planus
• Behcet's syndrome
• Recurrent aphthous ulcers
• Herpetic gingivostomatitis
• Granuloma annulare
LABORATORY: None
Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A
PATHOLOGICAL FINDINGS: A predominantly inflammatory pattern characterized by a lichenoid infiltrate which is of high density and rich in T-lymphocytes, and epidermal necrosis that mainly affects the basal layer
SPECIAL TESTS: N/A
IMAGING: N/A
DIAGNOSTIC PROCEDURES: Skin biopsy
TREATMENT APPROPRIATE HEALTH CARE: Care at home, unless mouth herpes precludes oral intake.
GENERAL MEASURES: • Treatment of any underlying or causative disease
• Withdrawal of any causative drugs
• For mild cases, symptomatic treatment is sufficient. For more severe cases, meticulous wound care and use of Burow's solution or Domeboro solution dressings.
• Oral lesions can be treated with mouthwashes with warm saline, or a solution of diphenhydramine, lidocaine (Xylocaine), and Kaopectate to provide symptomatic relief and oral hygiene, and to facilitate oral intake
SURGICAL MEASURES: N/A
ACTIVITY: As tolerated
DIET: As tolerated with increased fluid intake
PATIENT EDUCATION: Patients should be reassured that the disease is self-limited. Recurrences are possible. Encourage avoidance of any identified etiologic agent.
MEDICATIONS DRUG(S) OF CHOICE: The use of steroids is controversial. Patients who have herpes induced EM may benefit from acyclovir, and those who take acyclovir between episodes of recurrent herpetic EM get fewer episodes. Other causative infections should be treated. appropriately.
Contraindications: Some underlying infections or health problems such as diabetes may contraindicate the use of steroids
Precautions: Refer to manufacturer's profile of each drug
Significant possible interactions: Refer to manufacturer's profile of each drug
ALTERNATIVE DRUGS: None have been shown to be useful
FOLLOW UP PATIENT MONITORING: The disease is self limiting. Complications are rare. There is no mortality.
PREVENTION/AVOIDANCE: • Known or suspected etiologic agents should be avoided
• Acyclovir may help prevent herpes-related erythema multiforme
• Tamoxifen has been shown to prevent premenstrual related disease
POSSIBLE COMPLICATIONS: Corneal ulceration is a serious complication of Stevens-Johnson syndrome. It is not clear whether this can occur in true EM. While there may be complications of the underlying disease, there are no other complications of EM.
EXPECTED COURSE AND PROGNOSIS: • Rash evolves over 1-2 weeks and subsequently resolves within 2-3 weeks, generally without scarring or sequelae
• Following resolution there may be some post-inflammatory hyperpigmentation
• Risk of recurrence may be as high as 37%
MISCELLANEOUS ASSOCIATED CONDITIONS: Any of the infections or diseases listed under causes.
AGE-RELATED FACTORS:
Pediatric: More severe forms of the disease tend to occur in younger males. Rare under age 3 years.
Geriatric: Rare over age 50 years
Others: N/A
PREGNANCY: Reported as a possible etiologic condition
SYNONYMS: Erythema exudativum multiforme
ICD-9-CM: 695.1 erythema multiforme
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