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[先天性皮肤发育不全。2例报告]

[Aplasia cutis congenita. Report of 2 cases].

作者信息

Tincopa Wong O, Pelaez Gutierrez R, Melendez Guevara G, Paoli Razuri C, Sanchez Aznaran N

出版信息

Med Cutan Ibero Lat Am. 1986;14(3):199-204.

PMID:3526047
Abstract

In 1983, we had two cases of pediatric patients, which were observed in the Regional Hospital "Victor Lazarte Echegaray" of the Peruvian Institute of Social Security in Trujillo, these cases correspond to the syndrome of aplasia cutis congenita. In the first case, the defect was localized as a of ulcer in the right inferior member, which was affected from the third part distal of the thigh to the back part of the foot. In the second one, the lesion was in the middle line of the hairy skin, and it had the particularity of being associate to others congenital malformations. These cases are mentioned because of their rarity of presentation at a world level as well as their unknown occurrence in our country. Then we comment the clinic, histological characteristics, associate congenital defects, possible etiopathogenic mechanisms, differential diagnostic and its evolution and treatment.

摘要

1983年,我们在秘鲁社会保障局位于特鲁希略的“维克托·拉萨尔特·埃切加雷”地区医院观察到两例儿科患者,这些病例符合先天性皮肤发育不全综合征。在第一例中,缺损定位在右下肢体的一处溃疡,从大腿远端三分之一处至足部后部均受影响。在第二例中,病变位于有毛皮肤的中线,其特殊之处在于与其他先天性畸形相关联。提及这些病例是因为它们在世界范围内呈现罕见性,以及在我国其发病情况未知。随后我们对其临床症状、组织学特征、相关先天性缺陷、可能的病因发病机制、鉴别诊断及其演变和治疗进行了阐述。

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