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与烟雾病及烟雾综合征相关的 phakomatoses 和皮肤表现

Phakomatoses and cutaneous manifestations associated with moyamoya disease and syndrome.

作者信息

Tomaszewska Maja, Strzelczuk-Judka Lidia, Ostalska-Nowicka Danuta, Jończyk-Potoczna Katarzyna

机构信息

Department of Pediatric Radiology, Chair of General and Invasive Radiology, Poznan University of Medical Sciences, Poznan, Poland.

Department of Pediatric Cardiology and Nephrology, Poznan University of Medical Sciences, Poznan, Poland.

出版信息

Postepy Dermatol Alergol. 2020 Dec;37(6):831-835. doi: 10.5114/ada.2020.102092. Epub 2021 Jan 6.

Abstract

Phakomatoses are a group of neurocutaneous disorders whose origin is derived from the embryonic ectoderm. These disorders affect the central nervous system, the eyes, and the skin. This article presents phakomatoses and cutaneous manifestations associated with moyamoya disease and syndrome. Moyamoya disease is a progressive and occlusive disorder of the cerebral vasculature often presenting with particular phakomatoses. This article aims to reveal why patients with phakomatoses qualify for detailed neuroimaging.

摘要

phakomatoses是一组起源于胚胎外胚层的神经皮肤疾病。这些疾病会影响中枢神经系统、眼睛和皮肤。本文介绍了与烟雾病及烟雾综合征相关的phakomatoses及其皮肤表现。烟雾病是一种进行性的脑血管闭塞性疾病,常伴有特定的phakomatoses。本文旨在揭示为什么患有phakomatoses的患者需要进行详细的神经影像学检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/90e8/7874860/4d5761ef870d/PDIA-37-42819-g001.jpg

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