Abstract
This study summarizes current research and understanding of the pathogenesis of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) and provides an update on the treatment of these conditions in children. The association of specific human leukocyte antigen subtypes with SJS and TEN occurring in certain racial groups to specific drugs has led to recommendations on pretreatment testing. Several pathways have been postulated to lead to keratinocyte apoptosis in SJS and TEN. These include Fas-Fas ligand interaction, cytotoxic T-cell and natural killer-cell damage via perforin/granzyme B/granulysin and tumor necrosis factor-alpha. The use of intravenous immunoglobulins and systemic corticosteroids in TEN is still controversial, and more trials are needed to prove the efficacy of these agents. Newer agents such as cyclosporin, infliximab and plasmapheresis have shown promise in the treatment of SJS and TEN. As the pathogenesis of SJS and TEN is further unraveled, the emergence of newer therapeutic agents with more specific mechanisms of action may lead to improved survival in this oftentimes devastating disease.
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