1. Erythema multiforme minor is characterized by a skin rash, which may be accompanied by the involvement of one mucous membrane site.
2. Erythema multiforme major (Stevens-Johnson syndrome) is characterized by severe and extensive mucous membrane involvement and the involvement of multiple organ systems.
ETIOLOGY
Erythema multiforme appears to be a hypersensitivity reaction to drugs, infectious organisms, and other unknown entities. Causes include:
1. Drugs (especially aspirin, penicillins, sulfonamides, phenytoin, rifampin, and phenobarbital).
2. Infectious diseases, most commonly herpes simplex, Mycoplasma infection, Coxsackie and adenovirus infections, hepatitis B, and histoplasmosis.
3. Vaccines, including Bacille Calmette-Guérin and the poliomyelitis vaccine
4. Idiopathic (50% of cases)
CLINICAL FEATURES
The onset is sudden. Fever, malaise, and arthralgias are common. The lesions usually spare the trunk.
1. Dermal lesions: The rash may present as erythematous macules, papules, wheals, vesicles, or bullae. It appears mostly on the palms, soles, and dorsa of the extremities. The dermal lesions (“target lesions”) are papules or vesicles surrounded by a zone of normal skin and then a halo of erythema; they resemble a bull’s eye target.
2. Mucosal lesions: Hemorrhagic lesions can be found on the lips and oral mucosa.
3. A burning sensation is present on the skin and mucous membranes. Pruritus is absent.
DIFFERENTIAL DIAGNOSES
1. The dermal lesions must be differentiated from secondary syphilis, contact dermatitis, and meningococcemia.
2. The mucosal lesions must be differentiated from pemphigus and herpetic stomatitis.
EVALUATION
Diagnosis depends primarily on history and physical examination findings. Skin biopsy specimens show edema, extravasated erythrocytes, and necrolysis in the epidermis.
THERAPY
1. The cause should be treated (e.g., with antibiotics or termination of drug therapy) if it can be identified.
2. The use of systemic corticosteroids is controversial and has been associated with both remissions of the disease and with secondary, fatal, respiratory infections. If steroid treatment is used, prednisone (2 mg/kg/day) is given with subsequent tapering.
DISPOSITION
Patients with mild cases are treated as outpatients; patients with more severe mucous membrane involvement require hospitalization. Recurrent attacks lasting 2 to 4 weeks and usually occurring in the spring or autumn have been reported.
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