TY - CHAP M1 - Book, Section TI - Epidermal Necrolysis (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis) A1 - Mockenhaupt, Maja A1 - Roujeau, Jean-Claude A2 - Kang, Sewon A2 - Amagai, Masayuki A2 - Bruckner, Anna L. A2 - Enk, Alexander H. A2 - Margolis, David J. A2 - McMichael, Amy J. A2 - Orringer, Jeffrey S. Y1 - 2019 N1 - T2 - Fitzpatrick's Dermatology, 9e AB - AT-A-GLANCEEpidermal necrolysis is a rare life-threatening reaction mainly induced by medication.Widespread apoptosis of keratinocytes is provoked by the activation of a cell-mediated cytotoxic reaction and amplified by cytokines, mainly granulysin.Confluent purpuric and erythematous macules evolving to flaccid blisters and epidermal detachment often start on the upper trunk and spread to the limbs associated with mucous membrane involvement.Histopathology shows full-thickness necrosis of epidermis associated with mild mononuclear cell infiltrate.A dozen “high-risk” drugs account for half of cases.More than 20% of cases remain idiopathic or may be caused by infection.Early identification and withdrawal of suspect medication in drug-induced cases are essential for good patient outcomes.Treatment consists mainly of supportive care, but recent evidence suggests a significant benefit of immunomodulating treatment with cyclosporine.The death rate is high and increases with disease severity and age of the patient. The majority of survivors have long-lasting sequelae, needing systematic follow-up examinations. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=1162814448 ER -