儿童泛发性脓疱型银屑病发病机制及治疗进展
收稿日期: 2024-11-25
录用日期: 2025-01-07
网络出版日期: 2025-06-27
Progress in pathogenesis and treatment of pediatric generalized pustular psoriasis
Received date: 2024-11-25
Accepted date: 2025-01-07
Online published: 2025-06-27
泛发性脓疱型银屑病（GPP）是一种罕见的、复发性的全身性炎症性皮肤病。白细胞介素（IL）-36RN（IL36RN）是GPP最常见的致病基因。由IL-1/IL-36-趋化因子-中性粒细胞轴介导的先天性免疫在GPP的发病机制中起核心作用，肿瘤坏死因子-α（TNF-α）/IL-23/IL-17介导的适应性免疫也参与GPP发病。阿维A、甲氨蝶呤、环孢素和生物制剂是目前GPP的主要治疗药物。大部分生物制剂在儿童GPP仍属于超适应证使用。越来越多证据表明，阿达木单抗和司库奇尤单抗治疗儿童GPP已取得较好疗效。IL-36受体抑制剂佩索利单抗是GPP新的治疗靶点，为GPP治疗和预防带来新的希望。文章就儿童GPP的发病机制和治疗进展进行综述，为该病的临床诊治提供参考。
吕明君 , 罗文 , 杨锦向 , 梁键莹 , 姚志荣 . 儿童泛发性脓疱型银屑病发病机制及治疗进展[J]. 临床儿科杂志, 2025 , 43(7) : 556 -562 . DOI: 10.12372/jcp.2025.24e1264
Generalized pustular psoriasis (GPP) is a rare, recurrent systemic inflammatory skin disease. Interleukin (IL)-36RN (IL36RN) is the most common causative gene in GPP. Innate immunity mediated by IL-1/IL-36-chemokine-neutrophils plays a central role in the pathogenesis of GPP, and adaptive immunity mediated by tumor necrosis factor-α (TNF-α)/IL-23/IL-17 is also involved in GPP pathogenesis. At present, the main therapeutic agents for GPP are acitretin, methotrexate, cyclosporine and biologics. Most of the biologics are still used as over-indications in pediatric GPP. There is increasing evidence that adalimumab and secukinumab have achieved better efficacy in the treatment of GPP in children. Spesolimab, an IL-36 receptor inhibitor, is a new therapeutic target for GPP, bringing new hope for the treatment and prevention of GPP. This article reviews the pathogenesis and treatment progress of GPP in children, and provides reference for the clinical diagnosis and treatment of the disease.
Key words： generalized pustular psoriasis; pathogenesis; treatment progress; biologics; child
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