Nephrogenic fibrosing dermopathy
Nephrogenic fibrosing dermopathy
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DOI：
10.1097/00000372-200110000-00001
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发表时间：
2001-10-01
影响因子：
1.1
通讯作者：
LeBoit, PE
中科院分区：
文献类型：
--
作者：
Cowper, SE;Su, LD;LeBoit, PE
本报告详细介绍了肾脏疾病患者中最近出现的一种独特的纤维化疾病的组织病理学结果，受影响的患者最初在单一机构的肾移植受者中确定，但后来在其他中心确定了病例，包括因各种不同肾脏疾病接受肾透析的患者。皮肤改变以四肢和躯干的硬结斑和丘疹为主。在硬化性粘液水肿中观察到的全身性结果（在某些方面与该疾病相似）不存在。通过常规显微镜检查，发现从早期病变中真皮成纤维细胞的非常细微的增殖，到完全发育病例中成纤维细胞和树突状细胞的大量增殖。周围有裂缝的厚胶原束是一个突出的发现，真皮粘蛋白和弹性纤维的可变增加通常是明显的特殊染色。CD-34阳性真皮树突状细胞丰富，树突状突起与弹性纤维对齐，并在密集的网络中围绕胶原束。因子XIIIa。CD-68阳性单核和多核细胞也以增加的数量存在。电子显微镜突出增加的弹性纤维紧密贴附树突状细胞的过程。通常累及整个真皮，梭形细胞、胶原蛋白、粘蛋白和弹性纤维穿过皮下组织增加。沿着脂肪小叶的隔。在某些情况下，该过程类似于肉瘤的组织病理学检查。最近出现的这种情况和在特定透析中心的病例明显聚集，最初表明可能存在感染和/或毒性因子。然而，迄今为止，尚未发现任何此类物质。我们建议术语“肾源性纤维化皮肤病（NFD）”，直到一个特定的原因可以确定。
This report details the histopathologic findings in a unique fibrosing disorder that recently emerged among patients with renal disease, The affected patients were initially identified among recipients of renal transplants at a single institution, but later cases at other centers were identified, and included patients receiving renal dialysis for a variety of different kidney diseases. The cutaneous chan-es consisted largely of indurated plaques and papules on the extremities and trunk. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent. By routine microscopy, the findings range from a very subtle proliferation of dermal fibroblasts in early lesions, to a florid proliferation of fibroblasts and dendritic cells in fully developed cases. Thick collagen bundles with surrounding clefts are a prominent finding, and a variable increase in dermal mucin and elastic fibers was usually evident with special stains. CD-34 positive dermal dendrocytes were floridly abundant, with dendritic processes aligned with elastic fibers and around collagen bundles in a dense network. Factor XIIIa. and CD-68 positive mono- and multinucleated cells are also present in increased numbers. Electron microscopy highlighted increased elastic fibers closely apposed to dendritic cell processes. The entire dermis was commonly involved, with increased spindle cells, collagen, mucin, and elastic fibers extending through the subcutis. along the septa of fatty lobules. In some instances, the process resembled a sarcoma on histopathologic examination. The recent emergence of this condition and the apparent clustering of cases in specific dialysis centers initially suggested a possible infectious and/or toxic agent. To date, however, no such agent has been identified. We propose the term "nephrogenic fibrosing dermopathy (NFD)" until a specific cause can be identified.