Saito Yuki, Abe Riichiro
Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Curr Opin Allergy Clin Immunol. 2023 Aug 1;23(4):271-278. doi: 10.1097/ACI.0000000000000914. Epub 2023 Jun 6.
Recent studies have been clarifying the pathogenesis and early diagnostic markers of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Additionally, the efficacy of tumor necrosis factor alpha inhibitors is attracting attention. This review provides) recent evidence for the diagnosis and management of SJS/TEN.
Risk factors for the development of SJS/TEN have been identified, particularly the association between HLA and the onset of SJS/TEN with specific drugs, which has been intensively studied. Research on the pathogenesis of keratinocyte cell death in SJS/TEN has also progressed, revealing the involvement of necroptosis, an inflammatory cell death, in addition to apoptosis. Diagnostic biomarkers associated with these studies have also been identified.
The pathogenesis of SJS/TEN remains unclear and effective therapeutic agents have not yet been established. As the involvement of innate immunity, such as monocytes and neutrophils, in addition to T cells, has become clear, a more complex pathogenesis is predicted. Further elucidation of the pathogenesis of SJS/TEN is expected to lead to the development of new diagnostic and therapeutic agents.
近期研究一直在阐明史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）的发病机制及早期诊断标志物。此外，肿瘤坏死因子α抑制剂的疗效也备受关注。本综述提供了SJS/TEN诊断和管理的最新证据。
已确定SJS/TEN发生的危险因素，尤其是HLA与特定药物引发SJS/TEN之间的关联，对此已进行了深入研究。SJS/TEN中角质形成细胞死亡发病机制的研究也取得进展，除凋亡外，还揭示了坏死性凋亡（一种炎性细胞死亡）的参与。与这些研究相关的诊断生物标志物也已确定。
SJS/TEN的发病机制仍不清楚，尚未确立有效的治疗药物。由于除T细胞外，单核细胞和中性粒细胞等固有免疫的参与已明确，预计发病机制会更加复杂。进一步阐明SJS/TEN的发病机制有望推动新诊断和治疗药物的开发。
