自身炎症性皮肤病

doi:10.35541/cjd.20201253

中华皮肤科杂志 ›› 2023, Vol. 56 ›› Issue (8): 794-799.doi: 10.35541/cjd.20201253

自身炎症性皮肤病

贾韬宋相瑾耿松梅

西安交通大学第二附属医院皮肤病院，西安 710004

收稿日期:2020-12-30 修回日期:2021-02-13 发布日期:2023-08-07
通讯作者: 耿松梅 E-mail:gsm312@yahoo.com
基金资助:
陕西省重点研发计划项目（2020GXLH-Y-022）

Autoinflammatory dermatosis

Jia Tao, Song Xiangjin, Geng Songmei

Department of Dermatology, The Second Affiliated Hospital of Xi′an Jiaotong University, Xi′an 710004, China

Received:2020-12-30 Revised:2021-02-13 Published:2023-08-07
Contact: Geng Songmei E-mail:gsm312@yahoo.com
Supported by:
Key Research and Development Program of Shaanxi Province of China（2020GXLH-Y-022）

摘要/Abstract

摘要： 【摘要】自身炎症性疾病（AID）是一组由固有免疫细胞介导的异常炎症反应性疾病，具有一定的遗传易感性，包含单基因和多基因突变。AID临床表现多样，常累及多系统，具有周期性发作、间歇性症状缓解的特点，皮肤是主要受累的器官之一。由于AID涉及多种突变基因，并有一定的不确定性，从基因分类不利于临床辨识和治疗方案的选择。随着对AID发病机制研究的深入，研究人员发现具有类似皮损的AID也有类似的炎症通路，可采用类似的治疗方案。因此本文从皮损表现分类阐述主要的AID，总结其免疫机制及组织病理特征的共性，探讨可能有效的治疗方案和干预措施，以期对临床诊治有所裨益。

关键词: 遗传性自身炎症性疾病, 分类法, 皮肤表现

Abstract: 【Abstract】 Autoinflammatory diseases （AIDs） are a group of abnormal inflammatory diseases mediated by innate immune cells with a certain hereditary susceptibility, including monogenic AIDs and polygenic AIDs. AIDs are characterized by various clinical manifestations, periodic attacks and intermittent symptom relief, multiple systems are often involved, and skin is one of the predominantly involved organs. Due to genetic diversity and uncertainty in AIDs, gene-based classification is unhelpful in clinical recognitoin and selection of therapeutic strategies. With in-depth study of the pathogenesis of AIDs, researchers have found overlapping inflammatory pathways in AIDs sharing common skin lesions, which may be treated with similar strategies. This review describes classification of AIDs according to features of skin lesions, summarizes commonalities in their immune mechanisms and histopathological features, and discusses possible effective treatment regimens and interventions, in order to facilitate the clinical diagnosis and treatment.

Key words: Hereditary autoinflammatory diseases, Classification, Skin manifestations

贾韬宋相瑾耿松梅. 自身炎症性皮肤病[J]. 中华皮肤科杂志, 2023,56(8):794-799. doi:10.35541/cjd.20201253

Jia Tao, Song Xiangjin, Geng Songmei. Autoinflammatory dermatosis[J]. Chinese Journal of Dermatology, 2023, 56(8): 794-799.doi:10.35541/cjd.20201253

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自身炎症性皮肤病

Autoinflammatory dermatosis

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