Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

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Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening diseases that affect the skin and mucous membranes. They are usually caused by medications, especially antibiotics, antiepileptic drugs, and pain relievers, although infections can also trigger them. SJS involves less than 10% skin detachment, TEN involves more than 30%, and intermediate cases are called SJS/TEN overlap.

A person with SJS often encounters primary care providers who have limited understanding of this life-threatening illness and may prescribe inappropriate medications. 

The disease develops through a severe immune reaction in which cytotoxic T cells and natural killer cells attack keratinocytes, leading to widespread skin cell death. Several pathways are involved, including granzyme, Fas/FasL, perforin, and inflammatory cytokines such as TNF-α, IL-6, and IFN-γ. Genetic susceptibility also plays an important role, as certain HLA alleles are linked to higher risk in specific populations.

Early diagnosis is essential. Biomarkers such as granulysin, RIP3, galectin-7, CCL-27, and IL-15 may help detect the disease and assess severity. Identifying the causative drug is also critical, and tools such as the ALDEN score and Naranjo score are commonly used. Disease severity is usually assessed with SCORTEN, while ABCD-10 and inflammatory markers such as NLR may also provide prognostic information.

The first step in management is immediate withdrawal of the suspected drug, followed by intensive supportive care. This includes fluid and electrolyte replacement, wound and mucosal care, pain control, nutritional support, and careful monitoring for complications. Infection is a major cause of death because the damaged skin barrier makes patients highly vulnerable to bacterial invasion. Prophylactic antibiotics are generally not recommended, as they may worsen outcomes.

SJS/TEN can also affect internal organs. Common complications include lung injury, acute kidney failure, liver damage, gastrointestinal bleeding, and ocular disease. Eye involvement is especially important because it may lead to long-term visual impairment, so early ophthalmologic care and long-term follow-up are recommended.

Systemic treatment remains controversial because strong evidence from large clinical trials is limited. Corticosteroids, intravenous immunoglobulin (IVIG), cyclosporine, TNF-α inhibitors, and plasmapheresis have all shown benefit in some studies, but results remain inconsistent. New therapies, including JAK inhibitors and FasL-targeted treatments, are being explored as promising future options.

In conclusion, SJS and TEN are severe adverse reactions with high mortality and significant long-term complications. Early recognition, prompt discontinuation of the offending drug, supportive care, and multidisciplinary management are the foundations of treatment. Continued research is needed to improve diagnosis, risk prediction, and targeted therapy.

 

Research
Published: 05 June 2025

Recent developments in the research of Stevens-Johnson syndrome and toxic epidermal necrolysis: pathogenesis, diagnosis and treatment (Additional references in the link)
https://link.springer.com/article/10.1186/s40001-025-02664-7#:~:text=Studies%20have%20shown%20that%20the%20levels%20of,and%20is%20positively%20correlated%20with%20disease%20severity.

© 2000-2030 Sieglinde W. Alexander. All writings by Sieglinde W. Alexander have a five-year copyright. Library of Congress Card Number: LCN 00-192742 ISBN: 0-9703195-0-9 


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