IVL BioService, Saint-Barthélémy, Le Gua, France.
J Invest Dermatol. 2013 Feb;133(2):489-98. doi: 10.1038/jid.2012.330. Epub 2012 Sep 20.
Toxic epidermal necrolysis (TEN) is a severe immune-mediated adverse cutaneous drug eruption characterized by rapid and extensive epithelial cell death in the epidermis and mucosae. The molecular events leading to this often fatal condition are only partially understood, but evidence suggests a dual mechanism implicating a "drug"-specific immune response on one side and the onset of target cell death by proapoptotic molecules including FasL on the other side. Herein, we describe a potential molecular bridge between these two events that involves inducible nitric oxide synthase (iNOS), which is highly upregulated in the skin of TEN patients. We show that activated T cells secrete high amounts of tumor necrosis factor-α (TNF-α) and IFN-γ, and that both cytokines lead to increased expression and activity of keratinocyte iNOS. A similar observation has been made with drug-specific T lymphocytes from a TEN patient exposed to the culprit drug. The resulting increase in nitric oxide significantly upregulates keratinocyte FasL expression, resulting in Fas- and caspase-8-mediated keratinocyte cell death. Taken together, our data suggest that T-lymphocyte activation by drugs in TEN patients may indirectly lead to FasL-mediated keratinocyte apoptosis, via a molecular bridge involving TNF-α, IFN-γ, and iNOS.
中毒性表皮坏死松解症(TEN)是一种严重的免疫介导的皮肤药物反应,其特征在于表皮和粘膜上皮细胞的快速和广泛死亡。导致这种情况的分子事件只有部分被理解,但有证据表明,双机制涉及一侧的“药物”特异性免疫反应,另一侧包括 FasL 在内的促凋亡分子导致靶细胞死亡。在此,我们描述了涉及诱导型一氧化氮合酶(iNOS)的这两个事件之间的潜在分子桥梁,iNOS 在 TEN 患者的皮肤中高度上调。我们表明,活化的 T 细胞分泌大量肿瘤坏死因子-α(TNF-α)和 IFN-γ,并且这两种细胞因子导致角质形成细胞 iNOS 的表达和活性增加。从接触致病药物的 TEN 患者的特异性 T 淋巴细胞中也观察到了类似的观察结果。由此产生的一氧化氮增加显著上调角质形成细胞 FasL 的表达,导致 Fas 和胱天蛋白酶-8 介导的角质形成细胞死亡。总之,我们的数据表明,TEN 患者的 T 淋巴细胞被药物激活可能通过涉及 TNF-α、IFN-γ 和 iNOS 的分子桥梁,间接导致 FasL 介导的角质形成细胞凋亡。