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伴有弹性假黄瘤样改变的肾源性纤维化皮肤病和钙化防御

Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes.

作者信息

Lewis Kevan G, Lester Brian W, Pan Teddy D, Robinson-Bostom Leslie

机构信息

Department of Dermatology, Brown Medical School, Providence, RI 02903, USA.

出版信息

J Cutan Pathol. 2006 Oct;33(10):695-700. doi: 10.1111/j.1600-0560.2006.00490.x.

DOI:10.1111/j.1600-0560.2006.00490.x
PMID:17026522
Abstract

Nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.

摘要

肾源性纤维化皮肤病(NFD)和钙化防御是与慢性肾病相关的罕见病症。在NFD和钙化防御中已观察到组织病理学变化,包括营养不良性真皮钙化,常与弹性纤维相关。一种模仿弹性假黄瘤(PXE)的真皮弹性纤维钙化模式先前已作为钙化防御情况下的偶然发现被报道。然而,尽管NFD和钙化防御都与肾脏疾病及异常钙沉积有关,但它们是具有不同临床和组织病理学特征的独立病理过程。通过介绍一名同时符合NFD和钙化防御的临床及组织病理学标准且组织学特征模仿PXE的患者病例,对两者的诊断标准进行了综述。

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