Le Stephanie T, Marusina Alina I, Merleev Alexander A, Kirane Amanda, Kruglinskaya Olga, Kunitsyn Andrey, Kuzminykh Nikolay Yu, Xing Xianying, Li Sophie Y, Liakos William, Kahlenberg J Michelle, Gompers Andrea, Downing Lauren, Marella Sahiti, Billi Allison C, Harms Paul W, Tsoi Lam C, Brüggen Marie-Charlotte, Adamopoulos Iannis E, Gudjonsson Johann E, Maverakis Emanual
Department of Dermatology, and.
Department of Surgery, University of California, Davis, Sacramento, California, USA.
JCI Insight. 2025 Feb 24;10(4):e178766. doi: 10.1172/jci.insight.178766.
Necrobiosis is a histologic term used to describe abnormal deposits of "degenerating" collagen within the skin. It can be found as an incidental finding in various granulomatous conditions, but is a hallmark of necrobiosis lipoidica (NL) and necrobiotic xanthogranuloma (NXG). There is limited prior research on necrobiosis. Here, we employed single-cell analysis of lesional and nonlesional skin to study the pathophysiology of necrobiosis. Our findings demonstrate that necrobiotic lesional skin is characterized by SPP1hi macrophages expressing MARCO; NKG7-expressing effector CD8+ T cells coexpressing CCL5, IFNG, GZMs, and PRF1; CCL5hi fibroblasts coexpressing CXCL9, diverse collagens (e.g., COL4A4, COL11A1, COL8A1), and TIMP1; and IGHM-expressing plasma cells. Integrative analysis of signaling ligands and receptor expression identified strong cell-cell communication between NKG7+ T cells, CCL5hi fibroblasts, and SPP1-expressing macrophages. In contrast, these cell populations were not dominant features of systemic sclerosis, another collagen deposition disease. Furthermore, although SPP1-expressing macrophages were detectable in sarcoidosis, IFNG-expressing T cells were a more defining feature of sarcoidosis compared with NL and NXG. From these findings, we speculate that necrobiosis results from the deposition of diverse collagens and ECM proteins through a process driven by CCL5-expressing fibroblasts and SPP1-expressing macrophages.
渐进性坏死是一个组织学术语,用于描述皮肤内“退化”胶原蛋白的异常沉积。它可在各种肉芽肿性疾病中偶然发现,但却是脂性渐进性坏死(NL)和渐进性坏死性黄色肉芽肿(NXG)的标志。此前关于渐进性坏死的研究有限。在此,我们对病变皮肤和非病变皮肤进行单细胞分析,以研究渐进性坏死的病理生理学。我们的研究结果表明,渐进性坏死性病变皮肤的特征是表达MARCO的SPP1高巨噬细胞;共表达CCL5、IFNG、颗粒酶(GZMs)和穿孔素(PRF1)的表达NKG7的效应性CD8 + T细胞;共表达CXCL9、多种胶原蛋白(如COL4A4、COL11A1、COL8A1)和金属蛋白酶组织抑制因子1(TIMP1)的CCL5高成纤维细胞;以及表达免疫球蛋白M(IGHM)的浆细胞。对信号配体和受体表达的综合分析确定了NKG7 + T细胞、CCL5高成纤维细胞和表达SPP1的巨噬细胞之间存在强烈的细胞间通讯。相比之下,这些细胞群不是另一种胶原蛋白沉积疾病——系统性硬化症的主要特征。此外,尽管在结节病中可检测到表达SPP1的巨噬细胞,但与NL和NXG相比,表达IFNG的T细胞是结节病更具特征性的表现。基于这些发现,我们推测渐进性坏死是由表达CCL5的成纤维细胞和表达SPP1的巨噬细胞驱动的过程中多种胶原蛋白和细胞外基质蛋白沉积所致。