Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management.

Robert Frantz,Kiran Motaparthi,Simo Huang,Abhirup Are

doi:10.3390/medicina57090895

Robert Frantz, Kiran Motaparthi + Show 2 more

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https://doi.org/10.3390/medicina57090895

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Abstract

Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. They are associated with significant morbidity and mortality, and early diagnosis and treatment is critical in achieving favorable outcomes for patients. In this scoping review, Excerpta Medica dataBASE and PubMed were searched for publications that addressed recent advances in the diagnosis and management of the disease. Multiple proteins (galectin 7 and RIP3) were identified that are promising potential biomarkers for SJS/TEN, although both are still in early phases of research. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN. Due to the rare nature of the disease, there is a lack of prospective, randomized controlled trials and conducting these in the future would provide valuable insights into the management of this disease.

Highlights

Academic Editor: Mauro SalvatoreAlessandro AlaibacReceived: 19 July 2021Accepted: 24 August 2021Published: 28 August 2021Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations.Licensee MDPI, Basel, Switzerland.Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are dermatologic emergencies characterized by widespread epidermal necrolysis and sloughing.They are considered to have the same pathophysiology and are classified based on body surface area (BSA) involved (Table 1) [1]
SJS/TEN can be distinguished from staphylococcal scalded skin syndrome (SSSS) by the level of epidermal detachment, which is subcorneal in SSSS and at the dermo-epidermal junction in SJS/TEN
The authors reported an standardized mortality ratio (SMR) of 0.320, indicating a survival benefit in patients treated with cyclosporine

Summary

Introduction

Academic Editor: Mauro SalvatoreAlessandro AlaibacReceived: 19 July 2021Accepted: 24 August 2021Published: 28 August 2021Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations.Licensee MDPI, Basel, Switzerland.Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are dermatologic emergencies characterized by widespread epidermal necrolysis and sloughing.They are considered to have the same pathophysiology and are classified based on body surface area (BSA) involved (Table 1) [1]. Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are dermatologic emergencies characterized by widespread epidermal necrolysis and sloughing. They are considered to have the same pathophysiology and are classified based on body surface area (BSA) involved (Table 1) [1]. These are rare diseases and reported incidence rates vary by location. Frey et al [2] reported an incidence of 5.76 cases of SJS/TEN per million persons per year in the UK from 1995–2013. Hsu et al [3] reported 9.2, 1.6, and

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