U.K. Guidelines for the Management of Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis in Adults 2016

D. Creamer; S.A. Walsh; P. Dziewulski; L.S. Exton; H.Y. Lee; J.K.G. Dart; J. Setterfield; C.B. Bunker; M.R. Ardern-Jones; K.M.T. Watson; G.A.E. Wong; M. Philippidou; A. Vercueil; R.V. Martin; G. Williams; M. Shah; D. Brown; P. Williams; M.F. Mohd Mustapa; C.H. Smith

Disclosures

The British Journal of Dermatology. 2016;174(6):1194-1227. 

In This Article

Purpose and Scope

The overall objective of the guidelines is to provide up-to-date, evidence-based recommendations for the diagnosis and management of the full spectrum of Stevens–Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) and SJS–TEN overlap in adults during the acute phase of the disease.

The document aims to: (i) offer an appraisal of all relevant literature up to February 2016, focusing on any key developments; (ii) address important, practical clinical questions relating to the primary guideline objective, i.e. accurate diagnosis and identification of cases and suitable treatment; (iii) provide guideline recommendations; and (iv) discuss areas of uncertainty, potential developments and future directions

SJS/TEN is rare and few healthcare professionals are confident in the recognition and management of the disorder. There is widely divergent practice among different specialities and healthcare settings, and limited information on outcomes. These guidelines aim to provide recommendations on the diagnosis and management of SJS/TEN, to inform clinical decision-making and, when justified by evidence, to standardize practice. The breadth of this document should be sufficient to assist clinicians of all relevant specialities in the management of patients with SJS/TEN. The recommendations will also inform pathways of care to optimize healthcare delivery and highlight key areas of uncertainty for future research.

In these guidelines, the term SJS/TEN encompasses the full spectrum of the disease, i.e. SJS, TEN and SJS–TEN overlap (see section 'What are the recognized clinical phenotypes in Stevens–Johnson syndrome/toxic epidermal necrolysis?' for clinical definition of the separate entities). The guideline is presented as a detailed review with highlighted recommendations for practical use (see section 'Summary'), in addition to the development of a new patient information leaflet [available on the British Association of Dermatologists' (BAD) website, www.bad.org.uk]. Unless otherwise specified, recommendations apply to all forms of the disease.

Exclusions

This guideline does not cover paediatric patients. An addendum to this guideline addressing the needs of paediatric patients with SJS/TEN is planned. The evidence for treatment of children differs from that of adult patients, and will be considered separately.

The management of long-term sequelae of SJS/TEN is not considered in this document.

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