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Erythema Multiforme and Stevens Johnson Syndrome

Erythema multiforme (EM) is an acute hypersensitivity reaction characterized by distinctive skin lesions and mucous membrane involvement that has a spectrum of severity. It occurs in two forms: the more common "minor" type and the more severe "major" type, also called Stevens-Johnson syndrome (SJS). Sometimes EM includes toxic epidermal necrolysis (TEN) or Lyell disease. EM minor first was described completely by von Hebra in 1866; Stevens and Johnson described the major variant in 1922. EM occurs more often in males, and 20% to 50% of cases occur in the pediatric age group, although rarely in those younger than age 3 years. A winter predominance is Stevens Johnson syndrome, erythema multiforme, multiform.

The pathologic process responsible for EM is unknown. A review of the literature generates an extensive list of causative or inciting agents. Most frequently mentioned are sulfonamide antibiotics and anticonvulsants, both used commonly in pediatric practice. Malignancies, radiotherapy, autoimmune diseases, and

EM minor is characterized by a distinctive rash. Classically, the rash is an erythematous ring that has a dusky center--the target or iris lesion. The rash also may be macular, papular, and/or vesicular.

In contrast, Stevens-Johnson syndrome typically is preceded by a serious prodrome consisting of fever, myalgias, arthralgias, sore throat, and abdominal complaints. This is followed by rapid (hours to days) and widespread bullous eruption involving the extremities, trunk, and at least two mucous membranes. The total body surface area involved can be extensive, possibly leading to fluid and electrolyte disturbances and secondary infection. Lesions of the mouth are more impressive than in

The rash of TEN starts as a generalized sunburn-like erythema, which quickly progresses to full-thickness epidermal sloughing, sometimes secondary to trivial trauma (Nikolsky sign). Then

EM is a clinical diagnosis. The classic symmetrically distributed, target-lesion rash usually confirms the diagnosis. In contrast to urticaria, EM is rarely pruritic and the duration of the rash is longer. Other annular lesions usually are not symmetric and spare the mucous membranes. Mucous membrane

The treatment for EM minor is supportive for the mild symptomology. Any inciting agents should be discontinued or treated, if possible. Cohen suggests that cases secondary to HSV be treated with a 5-day course of acyclovir. Weston et al strongly discourage the use of steroids in EM minor because