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Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Marianne Lerch1, Carlo Mainetti2, Benedetta Terziroli Beretta-Piccoli3
1Allergy/Dermatology Unit, Department of Internal Medicine, Kantonsspital Winterthur, Winterthur, Switzerland.
Clinical Reviews in Allergy & Immunology
|December 1, 2017
Summary
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug reactions requiring immediate medical attention. Early recognition and treatment are vital for survival, with management focusing on drug withdrawal and supportive care.
Area of Science:
- Dermatology
- Pharmacology
- Immunology
Background:
- Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, life-threatening mucocutaneous reactions.
- These hypersensitivity reactions are typically triggered by medications, presenting with influenza-like symptoms followed by painful skin and mucous membrane lesions.
- Distinguishing between SJS, SJS/TEN overlap, and TEN is based on the percentage of skin detachment, with diagnosis confirmed by clinical assessment and histopathology.
Purpose of the Study:
- To provide an overview of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).
- To highlight the importance of early recognition and management of these medical emergencies.
- To discuss the causative agents, pathogenesis, complications, and current treatment strategies for SJS/TEN.
Main Methods:
- Review of current literature on Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).
- Analysis of diagnostic criteria, including clinical presentation and histopathology.
- Evaluation of causative drugs, risk factors, and pathogenetic mechanisms.
- Assessment of common complications and management protocols.
Main Results:
- Mortality rates for SJS/TEN have decreased due to improved management and the availability of severity assessment scores like SCORTEN.
- Commonly implicated drugs include anti-infective sulfonamides, anti-epileptic drugs, oxicam NSAIDs, allopurinol, nevirapine, and chlormezanone.
- Pathogenesis involves drug-specific T-cell mediated cytotoxicity and genetic factors (HLA-linked).
- Frequent complications include ocular, renal, cutaneous, septicemia, pulmonary, and hepatic involvement.
Conclusions:
- Prompt drug withdrawal and multidisciplinary supportive care are crucial for managing SJS/TEN.
- Systemic corticosteroids, immunoglobulins, and cyclosporine A are frequently employed treatments.
- Further research is needed to identify factors contributing to epidermal necrolysis, particularly in non-drug-induced forms.
Keywords:
Drug reactionErythema multiformeIntravenous immunoglobulinsStevens-Johnson syndromeToxic epidermal necrolysisMore Related Videos
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