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合并斯特奇-韦伯综合征和克-特综合征的色素血管性斑痣性错构瘤病

Phakomatosis pigmentovascularis presenting with sturge-weber syndrome and klippel-trenaunay syndrome.

作者信息

Sen Sumit, Bala Sanchaita, Halder Chinmay, Ahar Rahul, Gangopadhyay Anusree

机构信息

Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.

出版信息

Indian J Dermatol. 2015 Jan-Feb;60(1):77-9. doi: 10.4103/0019-5154.147801.

DOI:10.4103/0019-5154.147801
PMID:25657402
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4318068/
Abstract

Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

摘要

色素血管性斑痣性错构瘤病（PPV）是一种罕见的皮肤疾病，其特征为毛细血管畸形与其他色素痣并存。目前已描述了四种类型和两种亚型，其中“a”亚型仅表现为皮肤型，“b”亚型还伴有系统性关联，如斯特奇-韦伯综合征或克-特综合征。在此，我们报告一例患者，其患有葡萄酒色斑、太田痣、斯特奇-韦伯综合征和克-特综合征，这是一种罕见的组合。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/6bbabaf3bd6f/IJD-60-77-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/17ca473f34cd/IJD-60-77-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/29a00717f80a/IJD-60-77-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/e08818035cb4/IJD-60-77-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/6bbabaf3bd6f/IJD-60-77-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/17ca473f34cd/IJD-60-77-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/29a00717f80a/IJD-60-77-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/e08818035cb4/IJD-60-77-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d9/4318068/6bbabaf3bd6f/IJD-60-77-g004.jpg

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