INCONTINENTIA PIGMENTI

Cassidy and Allanson's Management of Genetic Syndromes Pub Date : 2019-11-20 DOI:10.1002/9781119142812.ch136

Dian Donnai, Elizabeth A. Jones

引用次数: 1

Abstract

Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines (BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS). Qeios · Definition, February 10, 2020

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色素失禁(IP)是一种罕见的x连锁显性多系统外胚层发育不良，通常在男性中是致命的，在女性新生儿中表现为沿Blashko线(BL)的大疱性皮疹，随后是疣状斑块，随着时间的推移演变为色素沉着的旋转模式。它的进一步特征是牙齿异常，脱发，指甲营养不良，偶尔影响视网膜和中枢神经系统(CNS)。Qeios·定义，2020年2月10日

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Cassidy and Allanson's Management of Genetic Syndromes

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