Recent Advances in the Understanding of Severe Cutaneous Adverse Reactions

N.R. Adler; A.K. Aung; E.N. Ergen; J. Trubiano; M.S.Y. Goh; E.J. Phillips

Disclosures

The British Journal of Dermatology. 2017;177(5):1234-1247. 

In This Article

Abstract and Introduction

Abstract

Introduction

Severe cutaneous adverse reactions (SCARs) encompass a heterogeneous group of delayed hypersensitivity reactions, which are most frequently caused by drugs. Our understanding of several aspects of SCAR syndromes has evolved considerably over the last decade. This review explores evolving knowledge of the immunopathogenic mechanisms, pharmacogenomic associations, in vivo and ex vivo diagnostics for causality assessment, and medication cross-reactivity data related to SCAR syndromes. Given the rarity and severity of these diseases, multidisciplinary collaboration through large international, national and/or multicentre networks to collect prospective data on patients with SCAR syndromes should be prioritized. This will further enhance a systematized framework for translating epidemiological, clinical and immunopathogenetic advances into preventive efforts and improved outcomes for patients.

Severe cutaneous adverse reactions (SCARs) encompass a heterogeneous group of delayed hypersensitivity reactions, most frequently caused by drugs, which are associated with significant morbidity and mortality.[1,2] SCARs include Stevens–Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS)/drug-induced hypersensitivity syndrome (DIHS or HSS) and acute generalized exanthematous pustulosis (AGEP).[3] The clinical, biochemical and histological characteristics of these syndromes are summarized in Table 1.

Our understanding of several aspects of SCAR syndromes has evolved considerably over the last decade. The recent 2016 U.K. guidelines on the management of SJS/TEN in adults highlighted many areas of evolving research.[4] The aim of this article is to provide a complementary review of emerging immunopathogenic mechanisms, established pharmacogenomic associations, in vivo and ex vivo causality assessment tools and medication cross-reactivity data related to SCAR syndromes.

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