Hayashi Ryota, Abe Riichiro
Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Curr Opin Allergy Clin Immunol. 2025 Aug 1;25(4):230-236. doi: 10.1097/ACI.0000000000001082. Epub 2025 Jun 12.
Pathogenesis of severe cutaneous adverse reaction (SCAR) including Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) and drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS) has been identified by recent studies. This review provides recent understanding of pathomechanisms of SCAR and how to manage the patients with SCAR.
Recent studies, including single-cell data, identified key signaling pathways and immune phenotypes in SCAR. These studies have highlighted potential treatments, such as TNF-α inhibitors and JAK inhibitors. Moreover, severity score of DIHS/DRESS and many biomarkers for SCAR are provided.
The pathogenesis of SCAR remains unclear, and most effective therapeutic strategy has not yet been established. However, the pathogenesis of keratinocyte cell death in SJS/TEN, which is the most critical phenomenon, has been established. The difference of immune profile between early and late stage have been suggested in DIHS/DRESS. Although new therapeutic options have been identified by resent studies, there is a lack of trial data for the efficiency of them. Further trials and studies of SCAR is expected to lead to the development of general effective treatment options for SCAR.
近期研究已明确了严重皮肤不良反应(SCAR)的发病机制,包括史蒂文斯-约翰逊综合征(SJS)/中毒性表皮坏死松解症(TEN)以及药物性超敏反应综合征/伴嗜酸性粒细胞增多和全身症状的药物反应(DIHS/DRESS)。本综述阐述了对SCAR发病机制的最新认识以及如何治疗SCAR患者。
包括单细胞数据在内的近期研究确定了SCAR中的关键信号通路和免疫表型。这些研究突出了潜在的治疗方法,如肿瘤坏死因子-α(TNF-α)抑制剂和Janus激酶(JAK)抑制剂。此外,还提供了DIHS/DRESS的严重程度评分以及许多SCAR的生物标志物。
SCAR的发病机制仍不清楚,尚未确立最有效的治疗策略。然而,已明确了SJS/TEN中角质形成细胞死亡这一最关键现象的发病机制。DIHS/DRESS中已提示了早期和晚期免疫特征的差异。尽管近期研究发现了新的治疗选择,但缺乏关于其疗效的试验数据。预计对SCAR进行进一步的试验和研究将促成开发出针对SCAR的通用有效治疗方案。