中国美容医学2017,Vol.26Issue(8):48-50,3.
Proteus综合征临床表现及病理分析
Analysis of Clinical Manifestations and Pathological Characteristics of Proteus Syndrome
李开征 1刘林嶓 1翟晓梅 1赵红雁1
作者信息
- 1. 郑州大学第一附属医院整形外科 河南 郑州 450052
- 折叠
摘要
Abstract
Objective To explore the characteristics of clinical manifestations and pathological characteristics of PS(Proteus syndrome). Methods Analysed the clinical and pathological data of 10 patients with Proteus syndrome who were characteristic of cerebriform nevi from 1996 to 2016, and to review pertinent literature. Results All patients were born withsome clinical manifestations, which were ingravescent and conspicuous at their puberty. These patients are characterized by cerebriform connective tissue nevi on head and limbs as well as systemic epidermal nevi, with or without asymmetric growth of bone and soft tissue,vascular malformation, facial abnormity (blepharoptosis of upper lid, hypophasis). Histopathologic examination demonstrated epithelial nests, strands and cords of pathological cells invading the deep dermis,which had no significant difference with the common nevocytes. Histopathology of PS is characterized by the hyperlasia of melanocyte and collagenoue fiber, desmocytic reduction. Conclusion Proteus syndrome is a rarely congenital and variable disorder with tissue asymmetrical and excessive growth. The accurate diagnosis of the disorder mainly depends on clinical manifestations.关键词
Proteus综合征/临床表现/病理学/诊断/鉴别诊断Key words
proteus syndrome/clinical manifestation/pathology/diagnosis/antidiastole分类
医药卫生引用本文复制引用
李开征,刘林嶓,翟晓梅,赵红雁..Proteus综合征临床表现及病理分析[J].中国美容医学,2017,26(8):48-50,3.