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Int. J. Mol. Sci. 2016, 17(12), 2135; doi:10.3390/ijms17122135

Review of Toxic Epidermal Necrolysis

1
Sydney Medical School—Northern, University of Sydney, 2065 Sydney, Australia
2
Sutton Arthritis Research Laboratory, Kolling Institute, University of Sydney, 2065 Sydney, Australia
3
Dermatology Department, Royal North Shore Hospital, 2065 Sydney, Australia
*
Author to whom correspondence should be addressed.
Academic Editor: Terrence Piva
Received: 14 November 2016 / Revised: 9 December 2016 / Accepted: 12 December 2016 / Published: 18 December 2016
(This article belongs to the Special Issue Inflammatory Skin Conditions)
View Full-Text   |   Download PDF [1338 KB, uploaded 21 December 2016]   |  

Abstract

Toxic epidermal necrolysis (TEN) is a rare but life threatening mucocutaneous reaction to drugs or their metabolites. It is characterised by widespread keratinocyte apoptosis and sloughing of the skin, erosions of the mucous membranes, painful blistering, and severe systemic disturbance. The pathophysiology of TEN is incompletely understood. Historically, it has been regarded as a drug-induced immune reaction initiated by cytotoxic lymphocytes via a human leukocyte antigen (HLA)-restricted pathway. Several mediators have been identified as contributors to the cell death seen in TEN, including; granulysin, soluble Fas ligand, perforin/granzyme, tumour necrosis factor-α (TNF-α), and TNF-related apoptosis-inducing ligand. Currently, granulysin is accepted as the most important mediator of T cell proliferation. There is uncertainty around the accepted management of TEN. The lack of definitive management guidelines for TEN is explained in part by the rarity of the disease and its high mortality rate, which makes it difficult to conduct randomised control trials on emerging therapies. Developments have been made in pharmacogenomics, with numerous HLA alleles identified; however, these have largely been ethnically specific. These associations have translated into screening recommendations for Han Chinese. View Full-Text
Keywords: inflammatory dermatoses; toxic epidermal necrolysis; drug reaction inflammatory dermatoses; toxic epidermal necrolysis; drug reaction
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Harris, V.; Jackson, C.; Cooper, A. Review of Toxic Epidermal Necrolysis. Int. J. Mol. Sci. 2016, 17, 2135.

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