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Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (Epithelial Necrolysis)

  • Jean-Claude Roujeau
Chapter

Abstract

Incidence of epithelial necrolysis (EN) is approximately two cases/million inhabitants/year. Adult women are overrepresented, and in childhood the sex ratio is more equal. The diagnosis of EN is often clinically apparent on examination of the skin and mucous membranes. Skin pathology helps differentiation from other blistering diseases. A prognosis score (SCORTEN) estimates the risk of death and the emergency of transfer to specialized wards (intensive care unit (ICU), Burn Units). A few “high-risk” medications cause close to 50% of cases (allopurinol, sulfamethoxazole, lamotrigine, carbamazepine, phenytoin, phenobarbital, oxicam class or Cox2 inhibitor class NSAIDs). Immediate withdrawal of suspect drug(s) and symptomatic therapeutic measures gives similar outcomes to those used in extensive burns. Cyclosporine A and etanercept likely decrease mortality.

Keywords

Epithelial necrolysis (EN) Drug-induced cytotoxicity Idiopathic cases High mortality Sequelae Symptomatic care Cyclosporine A 

Abbreviations

BSA

Body surface area

CI

Confidence interval

EN

Epithelial necrolysis

ICU

Intensive care unit

IVIg

Intravenous immunoglobulins

PNDS

Protocole National de Diagnostic et de Soins

PTSD

Post-traumatic stress disorder

SSSS

Staphylococcal Scalded Skin Syndrome

TEN

Toxic epidermal necrolysis

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Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Jean-Claude Roujeau
    • 1
  1. 1.Université Paris-Est-CréteilCréteilFrance

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