恶性萎缩性丘疹病/Köhlmeier-Degos 病于 1941 年由 Köhlmeier 在一份轶事病例报告中首次描述，该报告描述了一名年轻人出现广泛的多发性肠穿孔和丘疹性皮疹。 Köhlmeier-Degos 病是一种针对微脉管系统和动脉系统的独特血管病。其最典型的特征之一是涉及皮肤和胃肠道的离散多灶性凹陷瓷质病变。病理学发现是惊人的，可以大致分为血管性质的病理学结果与血管外基质产生的病理学结果，在广泛的血管外透明质酸和胶原蛋白沉积的背景下。动态进化形态不仅在临床上而且在组织学上都可以观察到。微血管改变在皮肤中尤其明显，其特征是内皮细胞坏死，随后内皮细胞脱离，并伴有管腔内纤维蛋白沉积，定义了血栓形成性微血管病，在后期病变中通常是少发性炎症。动脉病变非常独特，包括显着的新内膜增殖，以及由与血小板紧密混合的无定形胶原栓塞引起的血管腔闭塞。溶膜攻击复合物（即 C5b-9）介导的 I 型干扰素信号传导增强和内皮细胞损伤是血栓性微血管和闭塞性纤维化动脉病变演变的关键。我们描述了一例 Köhlmeier-Degos 病，该病是在使用戈利木单抗药物进行肿瘤坏死因子 (TNF)-α 抑制剂治疗时发生的。讨论了临床特征、光学显微镜检查结果以及基于 TNF-α 在控制 I 型干扰素反应中关键作用的病理生理学范式。版权所有 © 2024 作者。由 Wolters Kluwer Health, Inc. 出版

Malignant atrophic papulosis/Köhlmeier-Degos disease was first described in 1941 by Köhlmeier in an anecdotal case report that described a young man who presented with extensive multiple intestinal perforations and a papular skin rash. Köhlmeier-Degos disease represents a unique vasculopathy targeting both the microvasculature and the arterial system. One of its most characteristic features is reflected by the discrete multifocal depressed porcelain lesions involving the skin and gastrointestinal tract. The pathological findings are striking and can be broadly categorized into those that are vascular in nature versus extravascular matrix production in the context of extensive extravascular hyaluronic acid and collagen deposition. A dynamic evolutionary morphology is observed not only clinically but also histologically. The microvascular alterations are particularly evident in the skin and are characterized by endothelial cell necrosis with subsequent endothelial cell detachment accompanied by intraluminal fibrin deposition, defining a thrombogenic microangiopathy that in later stage lesions is typically pauci-inflammatory. The arterial lesions are very distinctive and include significant neointimal proliferation with vascular luminal occlusion by amorphous plugs of collagen intimately admixed with platelets. Pathogenetically enhanced type I interferon signaling and endothelial cell injury mediated by the membranolytic attack complex (ie, C5b-9) are key in the evolution of the thrombotic microvascular and obliterative fibrosing arteriopathic changes. We describe a case of Köhlmeier-Degos disease that developed in the setting of tumor necrosis factor (TNF)-alpha inhibitor therapy with the drug golimumab. The clinical features, light microscopic findings, and a pathophysiologic paradigm based on the critical role of TNF-alpha in controlling the type I interferon response are discussed.Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.