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广泛对称性先天性躯干皮肤发育不全,无纸样胎儿或胎盘异常的宏观证据。

Extensive symmetric truncal aplasia cutis congenita without fetus papyraceus or macroscopic evidence of placental abnormalities.

作者信息

Boente M del C, Frontini M del V, Acosta M I, Saleme C, Barrionuevo S, Asial R

机构信息

Departament of Dermatology, Hospital del Niño Jesús, Tucumán, Argentina.

出版信息

Pediatr Dermatol. 1995 Sep;12(3):228-30. doi: 10.1111/j.1525-1470.1995.tb00164.x.

Abstract

Aplasia cutis congenita is a rare disorder characterized by localized absence of skin at birth. Type V in Frieden's classification, which is associated with fetus papyraceous or placental infarcts, occurs as a large cutaneous defect on the trunk and extremities. The patient we report had a lesion affecting the trunk and extremities symmetrically, with no family history of the disorder or chromosomal abnormalities. In our opinion, despite the absence of fetus papyraceous or placental infarct, this patient's condition can be classified as type V.

摘要

先天性皮肤发育不全是一种罕见的疾病,其特征为出生时局部皮肤缺失。弗里登分类法中的V型与纸样胎儿或胎盘梗死相关,表现为躯干和四肢的大片皮肤缺损。我们报告的这名患者有一个对称累及躯干和四肢的病变,无该疾病家族史或染色体异常。我们认为,尽管没有纸样胎儿或胎盘梗死,该患者的病情仍可归类为V型。

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