中国实用儿科杂志

中国实用儿科杂志 ›› 2023, Vol. 38 ›› Issue (9): 695-700.DOI: 10.19538/j.ek2023090609

• 专题笔谈 • 上一篇    下一篇

特应性皮炎样皮肤表现的原发性免疫缺陷性疾病治疗进展

  

  1. 1.空军特色医学中心皮肤科，北京  100142 ；2.国家儿童医学中心  首都医科大学附属北京儿童医院皮肤科，北京  100045
  • 出版日期:2023-09-06 发布日期:2023-09-22
  • 通讯作者: 张斌，电子信箱：dr.binzhang@163.com
  • 基金资助:
    北京市自然科学基金（7222058）

Advances in the treatment of primary immunodeficiency diseases with atopic dermatitis-like skin manifestations

  1. *Department of Dermatology， Air Force Medical Center， PLA， Beijing 100142， China
  • Online:2023-09-06 Published:2023-09-22

摘要： 原发性免疫缺陷病（PID）是一组复杂的遗传性疾病，其特征为免疫系统功能缺陷。PID临床表现多样，如反复严重感染、自身免疫、自身炎症、肿瘤及特应性疾病等。特应性皮炎（AD）样表现是其常见的特征性皮肤损害之一。该文通过对AD皮肤表现PID发病机制和临床特征进行阐述，详细综述此类疾病治疗进展，旨在为此类疾病的治疗提供新的思路和选择。

关键词: 特应性皮炎, 原发性免疫缺陷病, 临床特征, 治疗进展

Abstract: Primary immunodeficiency diseases （PIDs） represent a group of complicated inherited disorders characterized by immune system defects. The clinical manifestations of PID are diverse， such as recurrent severe infections， autoimmunity， auto-inflammation， tumors and atopic diseases. Atopic dermatitis （AD）-like manifestation is one of common characteristic skin lesions of PID. This review aims to provide new ideas and options for the treatment of these diseases by elaborating on the pathogenesis and clinical features of PID with AD-like lesions and reviewing in detail the advances in the treatment of these diseases.

Key words: atopic dermatitis, primary immunodeficiency disease, clinical manifestations, treatment progress