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Journal ArticleDOI

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Update.

TLDR
This review summarizes up-to-date insights on SJS/TEN and describes a protocol for assessment and treatment, and it is hoped these suggested guidelines serve as a practical clinical tool in the management of SJS /TEN.
Abstract
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.

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Journal ArticleDOI

Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

TL;DR: The understanding of the pathogenesis of SJS/TEN has improved: drug-specific T cell-mediated cytotoxicity, genetic linkage with HLA- and non-HLA-genes, TCR restriction, and cytot toxicity mechanisms were clarified.
Journal ArticleDOI

SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation

Katie D. White, +50 more
TL;DR: The current state of knowledge and expert opinion related to SJS/TEN is summarized covering a broad spectrum of topics including epidemiology and pharmacogenomic networks; clinical management and complications; special populations such as pediatrics, the elderly, and pregnant women; regulatory issues and the electronic health record; new agents that cause SJS-TEN; pharmacogenomics and immunopathogenesis; and the patient perspective.
Journal ArticleDOI

Mechanisms of Severe Cutaneous Adverse Reactions: Recent Advances.

Teresa Bellón
- 01 Aug 2019 - 
TL;DR: Intensive research in recent years has led to major progress in understanding of the contribution of certain cell types and soluble mediators to the variability of SCAR phenotypes.
Journal ArticleDOI

Major psychological complications and decreased health-related quality of life among survivors of Stevens-Johnson syndrome and toxic epidermal necrolysis.

TL;DR: The long-term psychological complications and HRQOL of SJS/TEN survivors is characterized and 65% of participants had symptoms of post-traumatic stress and 29% even had total scores in keeping with clinical signs of possible PTSD.
References
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Journal ArticleDOI

Interferon-γ: an overview of signals, mechanisms and functions

TL;DR: The current understanding of IFN‐γ ligand, receptor, ignal transduction, and cellular effects with a focus on macrophage responses and to a lesser extent, responses from other cell types that influence macrophages function during infection are reviewed.
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Medical genetics: A marker for Stevens-Johnson syndrome

TL;DR: This paper showed that there is a strong association in Han Chinese between the human leukocyte antigen HLA-B*1502 and Stevens-Johnson syndrome induced by carbamazepine, a drug commonly prescribed for the treatment of seizures.
Journal ArticleDOI

Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme

TL;DR: This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents.
Journal ArticleDOI

Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin.

TL;DR: Antibodies present in pooled human intravenous immunoglobulins (IVIG) blocked Fas-mediated keratinocyte death in vitro and indicated that IVIG may be an effective treatment for toxic epidermal necrolysis of TEN.
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