Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis: An Update

Roni P. Dodiuk-Gad; Wen-Hung Chung; Laurence Valeyrie-Allanore; Neil H. Shear


Am J Clin Dermatol. 2015;16(6):475-493. 

In This Article

Abstract and Introduction


Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous reactions, predominantly drug induced. The mortality rates for SJS and TEN are as high as 30 %, and short- and long-term morbidities are very common. SJS/TEN is one of the few dermatological diseases that constitute a true medical emergency. Early recognition and prompt and appropriate management can be lifesaving. In recent years, our understanding of the pathogenesis, clinical presentation, and management of SJS/TEN has improved. Nevertheless, in 2015, there are still no internationally accepted management guidelines. This review summarizes up-to-date insights on SJS/TEN and describes a protocol for assessment and treatment. We hope these suggested guidelines serve as a practical clinical tool in the management of SJS/TEN. The classic manifestation of SJS/TEN consists of initial ''flu-like'' symptoms (malaise, fever, anorexia) in the prodromal phase, followed by cutaneous and mucous membrane (ocular, oral, and genital) inflammation and pain, and other systemic involvement. Symptoms usually begin 4–28 days after the onset of drug intake. Treatment is multidisciplinary and includes identification and withdrawal of the culprit drug, transfer to a specialist unit, supportive care, medical treatment, communication, and provision of appropriate information and emotional support.

1 Introduction

Stevens–Johnson syndrome (SJS) was first described by A. M. Stevens and F. C. Johnson in 1922.[1] The term ''toxic epidermal necrolysis'' (TEN) was coined in 1956 by A. Lyell.[2] Neither of those publications truly described what today is considered to be SJS or TEN. There are clinical and pathological variants of both SJS and TEN, but the terms are also used to define a condition that is a spectrum of disease from SJS to TEN. SJS and TEN represent different degrees of the same type of severe cutaneous adverse reaction. The mortality rates for SJS and TEN are high; that of TEN may approach 30 %.[3] In this manuscript, we refer to this specific disease spectrum as a single entity—namely, SJS/TEN.

In recent years, our understanding of the pathogenesis, clinical presentation, and management of SJS/TEN has been significantly enlightened. This review aims to summarize the most up-to-date insights.