Toxic epidermal necrolysis.
Toxic epidermal necrolysis (TEN) is an unpredictable, life-threatening drug reaction associated with a 30% mortality. Massive keratinocyte apoptosis is the hallmark of TEN. Cytotoxic T lymphocytes appear to be the main effector cells and there is experimental evidence for involvement of both the Fas-Fas ligand and perforin/granzyme pathways. Optimal treatment for these patients remains to be clarified. Discontinuation of the offending drug and prompt referral to a burn unit are generally agreed upon steps. Beyond that, however, considerable controversy exists. Evidence both pro and con exists for the use of IVIG, systemic corticosteroid, and other measures. There is also evidence suggesting that combination therapies may be of value. All the clinical data, however, is anecdotal or based on observational or retrospective studies. Definitive answers are not yet available. Given the rarity of TEN and the large number of patients required for a study to be statistically meaningful, placebo controlled trials are logistically difficult to accomplish. The absence of an animal model further hampers research into this condition. This article reviews recent data concerning clinical presentation, pathogenesis and treatment of TEN.


OBJECTIVE
At the conclusion of this learning activity, participants should have acquired a more comprehensive knowledge of our current understanding of the classification, clinical presentation, etiology, pathophysiology, prognosis, and treatment of TEN.
Pereira
Frederick A
FA
Department of Dermatology, Mount Sinai School of Medicine, New York, New York, USA. carozat@aol.com
Mudgil
Adarsh Vijay
AV
Rosmarin
David M
DM
D017209:Apoptosis / Q000502:physiology; D003937:Diagnosis, Differential; D053222:Fas Ligand Protein / Q000235:genetics; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D015603:Keratinocytes / Q000276:immunology; D008213:Lymphocyte Activation / Q000502:physiology; D010956:Plasmapheresis; D011110:Polymorphism, Genetic; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D013601:T-Lymphocytes / Q000502:physiology; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
D053222:Fas Ligand Protein; D016756:Immunoglobulins, Intravenous; D014409:Tumor Necrosis Factor-alpha