史蒂文斯-约翰逊综合征 (SJS) 和中毒性表皮坏死松解症 (TEN) 是危及生命的疾病，其特征是表皮和粘膜广泛脱落。这些严重疾病的死亡率很高，其发病机制仍不清楚。此外，SJS/TEN 的最佳治疗策略仍然是一个持续争论的话题。 SJS/TEN 的早期诊断具有挑战性，用于诊断或严重程度预测的可靠生物标志物尚未确立。某些药物，例如卡马西平和别嘌呤醇，已显示与特定人类白细胞抗原 (HLA) 类型密切相关。最近，人们探索了在施用这些药物之前进行 HLA 筛查以降低 SJS/TEN 发生率的潜在益处。 SJS/TEN 损伤中的表皮细胞死亡是由广泛的细胞凋亡引起的，主要通过 Fas-FasL 和穿孔素/颗粒酶途径。我们的研究结果表明，程序性坏死的一种形式坏死性凋亡也会导致表皮细胞死亡。单核细胞释放的膜联蛋白 A1 与甲酰肽受体 1 相互作用，诱导坏死性凋亡。一些生物标志物，如 CC 趋化因子配体 (CCL)-27、白介素-15、半乳糖凝集素-7、受体相互作用蛋白激酶 3 (RIP3) 和脂质运载蛋白-2，已被鉴定用于 SJS/TEN 的诊断和预后目的。建议对 SJS/TEN 进行支持治疗，但各种治疗方案（包括全身性皮质类固醇、静脉注射免疫球蛋白、环孢素和肿瘤坏死因子-α 拮抗剂）的疗效仍存在争议。最近的研究调查了肿瘤坏死因子-α 拮抗剂的潜在益处。在这篇综述中，我们讨论了 SJS/TEN 理解和管理方面的最新进展。版权所有 © 2024 The Chinese Medical Association，由 Wolters Kluwer, Inc. 根据 CC-BY-NC-ND 许可制作。

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions characterized by extensive detachment of the epidermis and mucous membranes. These severe disorders carry a high mortality rate, and their pathogenesis remains largely unclear. Furthermore, optimal therapeutic strategies for SJS/TEN remain a subject of ongoing debate. Early diagnosis of SJS/TEN is challenging, and reliable biomarkers for diagnosis or severity prediction have not been firmly established. Certain drugs, such as carbamazepine and allopurinol, have shown a strong association with specific human leukocyte antigen (HLA) types. Recently, the potential benefits of HLA screening prior to administering these drugs to reduce the incidence of SJS/TEN have been explored. Epidermal cell death in SJS/TEN lesions is caused by extensive apoptosis, primarily through the Fas-FasL and perforin/granzyme pathways. Our findings suggest that necroptosis, a form of programmed necrosis, also contributes to epidermal cell death. Annexin A1, released from monocytes, interacts with the formyl peptide receptor 1 to induce necroptosis. Several biomarkers, such as CC chemokine ligand (CCL)-27, interleukin-15, galectin-7, receptor-interacting protein kinases 3 (RIP3), and lipocalin-2, have been identified for diagnostic and prognostic purposes in SJS/TEN. Supportive care is recommended for treating SJS/TEN, but the efficacy of various therapeutic options-including systemic corticosteroids, intravenous immunoglobulin, cyclosporine, and tumor necrosis factor-α antagonists-remains controversial. Recent studies have investigated the potential benefits of tumor necrosis factor-α antagonists. In this review, we discuss recent advances in the understanding and management of SJS/TEN.Copyright © 2024 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license.