title: Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis.
abstract: Toxic epidermal necrolysis is a life-threatening, typically drug-induced mucocutaneous disease. It is clinically characterized as a widespread sloughing of the skin and mucosa, including both external and internal surfaces. Histologically, the denuded areas show full thickness epidermal necrosis. The pathogenic mechanism involves antigenic moiety/metabolite, peptide-induced T cell activation, leading to keratinocyte apoptosis through soluble Fas ligand, perforin/granzyme B, tumor necrosis factor-alfa, and nitric oxide. Recent studies have implicated granulysin in toxic epidermal necrolysis apoptosis and have suggested that it may be the pivotal mediator of keratinocyte death.
authors.affiliation: Dermatology, Preventive Medicine, and Pathology, Rutgers University New Jersey Medical School, Newark, New Jersey 07103-2714, USA. roschwar@cal.berkeley.edu
authors.forename: Robert A
authors.initials: RA
authors.lastname: Schwartz
authors.forename: Patrick H
authors.initials: PH
authors.lastname: McDonough
authors.forename: Brian W
authors.initials: BW
authors.lastname: Lee
mesh_terms: D017209:Apoptosis / Q000276:immunology; D001707:Biopsy, Needle; D002423:Cause of Death; D018450:Disease Progression; D004502:Education, Medical, Continuing; D019317:Evidence-Based Medicine; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015603:Keratinocytes / Q000276:immunology / Q000473:pathology; D008297:Male; D012307:Risk Factors; D012720:Severity of Illness Index; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology* / Q000401:mortality; D015996:Survival Rate
keywords: ALDEN; APC; BSA; CD40 ligand; CD40L; EM; Fas ligand; FasL; HLA; MHC; NF-κB; NK; NO; PBMC; SJS; Stevens–Johnson syndrome; TEN; algorithm for drug causality for epidermal necrolysis; antigen presenting cell; apoptosis; body surface area; drug eruption; erythema multiforme; granulysin; human leukocyte antigen; major histocompatibility complex; natural killer; nitric oxide; nuclear factor kappaB; peripheral blood mononuclear cell; sFasL; serum Fas ligand; toxic epidermal necrolysis