Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (Epithelial Necrolysis)

Abstract

Incidence of epithelial necrolysis (EN) is approximately two cases/million inhabitants/year. Adult women are overrepresented, and in childhood the sex ratio is more equal. The diagnosis of EN is often clinically apparent on examination of the skin and mucous membranes. Skin pathology helps differentiation from other blistering diseases. A prognosis score (SCORTEN) estimates the risk of death and the emergency of transfer to specialized wards (intensive care unit (ICU), Burn Units). A few “high-risk” medications cause close to 50% of cases (allopurinol, sulfamethoxazole, lamotrigine, carbamazepine, phenytoin, phenobarbital, oxicam class or Cox2 inhibitor class NSAIDs). Immediate withdrawal of suspect drug(s) and symptomatic therapeutic measures gives similar outcomes to those used in extensive burns. Cyclosporine A and etanercept likely decrease mortality.