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6例类脂蛋白沉积症的临床病理研究

A clinicopathologic study of six cases of lipoid proteinosis.

作者信息

Piérard G E, Van Cauwenberge D, Budo J, Lapière C M

机构信息

Department of Dermatopathology, University of Liège, Belgium.

出版信息

Am J Dermatopathol. 1988 Aug;10(4):300-5. doi: 10.1097/00000372-198808000-00004.

DOI:10.1097/00000372-198808000-00004
PMID:2458052
Abstract

We have reviewed the clinical and histological presentations of six cases of lipoid proteinosis. Deposits in the dermis were heterogeneous in nature and location. In the first stage of the disease's evolution, basement membrane components accumulate at the normal site of vascular, muscular, and epithelial basement membranes. Later on, lipids and amorphous material fill the superficial dermis, pushing apart the normal collagen bundles. Such deposits seem to be organized around preserved reticulin fibers. The amorphous material did not react with antibodies to collagen types I, III, IV, and V.

摘要

我们回顾了6例类脂蛋白沉积症的临床和组织学表现。真皮内的沉积物在性质和位置上是异质性的。在疾病演变的第一阶段,基底膜成分在血管、肌肉和上皮基底膜的正常部位积聚。随后,脂质和无定形物质填充浅表真皮,推开正常的胶原束。此类沉积物似乎围绕保留的网状纤维排列。无定形物质与I、III、IV和V型胶原抗体无反应。

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A clinicopathologic study of six cases of lipoid proteinosis.6例类脂蛋白沉积症的临床病理研究
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Three-dimensional imaging reveals major changes in skin microvasculature in lipoid proteinosis and lichen sclerosus.三维成像揭示了类脂蛋白沉积症和硬化性苔藓中皮肤微血管的主要变化。
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[A new case of Urbach-Wiethe disease. Electron-microscopy study].
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