中国麻风皮肤病杂志 ›› 2022, Vol. 38 ›› Issue (4): 256-259.doi: 10.12144/zgmfskin202204256

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毛囊角化病研究进展

张晓丽,刘毅   

  1. 中国医学科学院皮肤病医院临床药理及化妆品检测中心，南京， 210042
  • 出版日期:2022-04-15 发布日期:2022-02-10
  • 通讯作者: 刘毅，E-mail: liuyi@pumcderm.cams.cn

Update of Darier's disease

ZHANG Xiaoli, LIU Yi   

  1. Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical Colleges, Nanjing 210042, China
  • Online:2022-04-15 Published:2022-02-10
  • Contact: LIU Yi, E-mail: liuyi@pumcderm.cams.cn

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摘要： 毛囊角化病是一种常染色体显性遗传性皮肤病，临床表现为对称分布的褐色角化性丘疹、斑块，上覆油腻性鳞屑，好发于皮脂溢出部位。组织病理学特征为棘层松解性角化不良，本病与ATP2A2基因突变有关。目前治疗新进展包括新型药物、放射治疗、同种异体皮肤移植术和基因治疗等。

关键词: 毛囊角化病, 发病机制, 治疗进展

Abstract: Darier's disease (DD) is an autosomal dominant genodermatosis, with manifestations of brown keratotic papules and plaques distributed in the seborrhoeic regions, covered with greasy scales. Histopathology features are dyskeratosis and acantholysis. The gene mutation of ATP2A2 is associated with DD. The new therapy methods of DD include new drugs, radiation therapy, allograft skin and gene therapy.

Key words: Darier's disease, pathogenesis, treatment progress