王晨羽;龙笑;曾昂;黄久佐;王晓军;
WANG Chenyu;LONG Xiao;ZENG Ang;HUANG Jiuzuo;WANG Xiaojun;Department of Plastic and Reconstructive Surgery,Shanghai Ninth People's Hospital,Shanghai Jiao Tong University School of Medicine;Department of Plastic Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;State Key Laboratory of Complex Severe and Rare Disease,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;

• 1:上海交通大学医学院附属第九人民医院整复外科
• 2:中国医学科学院北京协和医院整形美容外科
• 3:中国医学科学院北京协和医院疑难重症及罕见病国家重点实验室

摘要(Abstract)：

局限性硬皮病是一种罕见的自身免疫性疾病，其皮肤纤维化和皮下组织萎缩可导致患者外貌受损，因此常需整形外科手术治疗。本文总结了局限性硬皮病的病理生理、术前评估、外科治疗进展与临床困境，并分享北京协和医院的诊治经验，以期为临床提供参考和借鉴。
Localized scleroderma is a rare auto-immune disorder characterized by inflammation and fibrosis of the skin and subcutaneous fat in the affected area, leaving aesthetic impairment to the patients.Such patients often seek plastic surgery for the treatment. This article summarizes the pathophysiology and preoperative evaluation of localized scleroderma, discusses the progress and clinical dilemma of its surgical treatment, and shares the opinions on the diagnosis and treatment of a single center in Peking Union Medical College Hospital.

关键词(KeyWords)： 局限性硬皮病;整形外科治疗;皮瓣移植;脂肪移植
localized scleroderma;plastic surgery treatment;flap reconstruction;fat grafting

Abstract:

Keywords:

基金项目(Foundation): 国家重点研发计划(2016YFA0101003,2020YFE0201600);; 中国医学科学院医学与健康科技创新工程“十四五”项目中国罕见病的精准诊疗研究(2021-I2M-1-003)~~

作者(Authors): 王晨羽;龙笑;曾昂;黄久佐;王晓军;
WANG Chenyu;LONG Xiao;ZENG Ang;HUANG Jiuzuo;WANG Xiaojun;Department of Plastic and Reconstructive Surgery,Shanghai Ninth People's Hospital,Shanghai Jiao Tong University School of Medicine;Department of Plastic Surgery,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;State Key Laboratory of Complex Severe and Rare Disease,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;

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