Toxic epidermal necrolysis.

Wolfram Hoetzenecker,Martin Glatz,Emmanuella Guenova,Peter Schmid-Grendelmeier,Antonio Cozzio,Tarun Mehra,Ieva Saulite,Lars E French

doi:10.12688/f1000research.7574.1

Wolfram Hoetzenecker, Martin Glatz + Show 6 more

Open Access

https://doi.org/10.12688/f1000research.7574.1

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Journal: F1000Research	Publication Date: May 20, 2016
Citations: 23	License type: CC BY 4.0

Affiliation: University Hospital of Zurich

Abstract

Toxic epidermal necrolysis (TEN) is a rare, life-threatening drug-induced skin disease with a mortality rate of approximately 30%. The clinical hallmark of TEN is a marked skin detachment caused by extensive keratinocyte cell death associated with mucosal involvement. The exact pathogenic mechanism of TEN is still uncertain. Recent advances in this field have led to the identification of several factors that might contribute to the induction of excessive apoptosis of keratinocytes. In addition, specific human leukocyte antigen types seem to be associated with certain drugs and the development of TEN. As well-controlled studies are lacking, patients are treated with various immunomodulators (e.g. intravenous immunoglobulin) in addition to the best supportive care.

Highlights

The exposure to drugs has increased with demographic shifts associated with a higher morbidity of the population
Toxic epidermal necrolysis (TEN) is a consequence of extensive keratinocyte cell death that results in the separation of significant areas of skin at the dermal-epidermal junction with the production of bullae followed by skin sloughing
This review aims to provide an up-to-date overview of TEN, emphasizing pathogenesis and immunopathology

Summary

Introduction

The exposure to drugs has increased with demographic shifts associated with a higher morbidity of the population. The skin lesion-associated erythematous plaques and widespread areas of epidermal detachment were referred to by Dr Lyell as necrolysis. He described an involvement of the mucous membranes as part of the syndrome and noted that there was very little inflammation in the dermis, a feature that was later referred to as “dermal silence”[3]. Preceding exposure to medications is reported in over 95% of patients with TEN, and a strong association between drug ingestion and cutaneous manifestation is observed in 80% of cases[1]. According to the latter two hypotheses, a pharmacological agent serving as the allergen would directly bind to specific HLA molecules and/or

C Altered peptide

Conclusion

Findings

Lyell A