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进行性心肌病性雀斑样痣:6例报告及1例尸检

Progressive cardiomyopathic lentiginosis: report of six cases and one autopsy.

作者信息

Ruiz-Maldonado R, Trevizo L, Tamayo L, de los Rios M F, Skurovich M, Carrillo J, Dominguez D, del Castillo V

出版信息

Pediatr Dermatol. 1983 Oct;1(2):146-53. doi: 10.1111/j.1525-1470.1983.tb01106.x.

Abstract

Progressive cardiomyopathic lentiginosis (PCL) is a syndrome with progressive multisystem abnormalities. Lentigines, the cutaneous hallmark, are usually present from early age and are of utmost diagnostic value. Six children with PCL are reported. The most constant diagnostic features in this age group were multiple cutaneous lentigines, structural cardiac anomalies, and electrocardiographic alterations. Less constant findings were neurosensory deafness, short stature, and osseous, ocular, and genitourinary abnormalities.

摘要

进行性心肌病性雀斑样痣综合征(PCL)是一种具有进行性多系统异常的综合征。雀斑样痣作为皮肤特征性表现,通常自幼出现,具有极高的诊断价值。本文报告了6例PCL患儿。该年龄组最常见的诊断特征为多发性皮肤雀斑样痣、心脏结构异常和心电图改变。较少见的表现为神经感觉性耳聋、身材矮小以及骨骼、眼部和泌尿生殖系统异常。

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