A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are very rare dermatologic disorders characterized by extensive detachment of the epidermis. Although some drugs, infectious agents, HLA genes, malignancy and connective tissue diseases are thought to play a role in the development...

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Main Authors: Ayca Kocaaga, Mustafa Kocaaga
Format: Article
Language:English
Published: KeAi Communications Co., Ltd. 2025-09-01
Series:Global Medical Genetics
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Online Access:http://www.sciencedirect.com/science/article/pii/S2699940425000554
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author Ayca Kocaaga
Mustafa Kocaaga
author_facet Ayca Kocaaga
Mustafa Kocaaga
author_sort Ayca Kocaaga
collection DOAJ
description Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are very rare dermatologic disorders characterized by extensive detachment of the epidermis. Although some drugs, infectious agents, HLA genes, malignancy and connective tissue diseases are thought to play a role in the development of the disease, the pathogenesis has not been fully elucidated. Type IV hypersensitivity reactions are thought to be the underlying cause of these diseases. Inflammatory changes such as purulent erosion, ulcers and crusting can be seen in the gastrointestinal system, urinary tract and genital mucosa. Some immunologic biyomarkers like as HLA-B* 1502, Granulysin and CCL-27 are used in clinical diagnosis, prognosis, treatment, and personalized new drug development that could further explain the pathogenesis of SJS and TEN. This review focuses on the pathophysiology of the SJS and TEN and presents it in light of current data on this subject. We recommend further in vivo and in vitro studies to unravel the underlying immunological mechanisms to determine appropriate intervention strategies.
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spelling doaj-art-ea57e97a7c234d5e9820e270019c8a392025-08-20T03:16:15ZengKeAi Communications Co., Ltd.Global Medical Genetics2699-94042025-09-0112310005410.1016/j.gmg.2025.100054A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysisAyca Kocaaga0Mustafa Kocaaga1Department of Medical Genetics, Eskişehir City Hospital, Eskisehir, Turkey; Correspondence to: Department of Medical Genetics, Eskişehir City Hospital, Eskişehir Şehir Hastanesi, 71 Evler Mahallesi, Çavdarlar Sokak, TR, 26080 Eskişehir, Odunpazarı, Turkey.Department of Medical Microbiology, Yunus Emre State Hospital, Uluönder mahallesi, Fevzi Çakmak Caddesi, No:1, TR 26190 Eskişehir, Tepebaşı, TurkeyStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are very rare dermatologic disorders characterized by extensive detachment of the epidermis. Although some drugs, infectious agents, HLA genes, malignancy and connective tissue diseases are thought to play a role in the development of the disease, the pathogenesis has not been fully elucidated. Type IV hypersensitivity reactions are thought to be the underlying cause of these diseases. Inflammatory changes such as purulent erosion, ulcers and crusting can be seen in the gastrointestinal system, urinary tract and genital mucosa. Some immunologic biyomarkers like as HLA-B* 1502, Granulysin and CCL-27 are used in clinical diagnosis, prognosis, treatment, and personalized new drug development that could further explain the pathogenesis of SJS and TEN. This review focuses on the pathophysiology of the SJS and TEN and presents it in light of current data on this subject. We recommend further in vivo and in vitro studies to unravel the underlying immunological mechanisms to determine appropriate intervention strategies.http://www.sciencedirect.com/science/article/pii/S2699940425000554AutoimmuneHLAImmunogeneticsStevens-Johnson syndromeToxic epidermal necrolysis
spellingShingle Ayca Kocaaga
Mustafa Kocaaga
A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis
Global Medical Genetics
Autoimmune
HLA
Immunogenetics
Stevens-Johnson syndrome
Toxic epidermal necrolysis
title A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis
title_full A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis
title_fullStr A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis
title_full_unstemmed A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis
title_short A review of the immunogenetics of Stevens-Johnson syndrome and toxic epidermal necrolysis
title_sort review of the immunogenetics of stevens johnson syndrome and toxic epidermal necrolysis
topic Autoimmune
HLA
Immunogenetics
Stevens-Johnson syndrome
Toxic epidermal necrolysis
url http://www.sciencedirect.com/science/article/pii/S2699940425000554
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