全身性脓疱型银屑病(3GPP)是银屑病的一个罕见的子集,涉及伴有炎症的无菌脓疱的发作,经常,系统参与。的炎症性质有可能导致严重的多系统并发症,包括高输出心力衰竭,感染,消化系统问题,和毁容或致命的急性耀斑发作。这种疾病在女性和亚洲人中的患病率较高。IL-1/IL-36炎症途径是3GPP病理学的一个重要方面。遗传突变,是与3GPP相关的包括白细胞介素36受体拮抗剂(IL36RN)的修饰,Caspase招募领域家庭成员14(CARD14),衔接子相关蛋白复合物1亚基Sigma3(AP1S3),髓过氧化物酶(MPO),和丝氨酸蛋白酶抑制剂A成员3(SERPINA3)基因。对3GPP的治疗指南并不根深蒂固。目前,只有一种3GPP特异性治疗,白细胞介素-36受体拮抗剂(IL-36Ra)spesolimab,已被批准在美国使用。目前正在开发其他抗IL-36途径疗法。其他治疗选择包括其他生物疗法,如IL-17抑制剂,IL-23抑制剂,和TNFα抑制剂。非生物治疗方案包括类维生素A,环孢菌素,和甲氨蝶呤.世界各地的治疗方案各不相同;大多数国家都使用类维生素A,环孢菌素,甲氨蝶呤作为一线非生物选择。中国和英国没有被批准使用的3GPP特异性生物疗法,虽然几种生物疗法被批准在日本使用。这篇综述旨在作为对当前的全球管理的更新,同时还包括疾病发病机制的相关方面。诊断,临床表现,组织病理学,病因学,和流行病学。
Generalized pustular psoriasis (GPP) is a rare subset of psoriasis involving episodes of sterile pustules accompanied by inflammation and, often, systemic involvement. The inflammatory nature of GPP has potential for severe multisystem complications including high-output cardiac failure, infections, digestive system issues, and disfiguring or lethal acute flare episodes. The disease tends to have higher prevalence in females and Asians. The IL-1/IL-36 inflammatory pathway is a critical facet of GPP\'s pathology. Genetic mutations that are associated with GPP include modifications of Interleukin 36 Receptor Antagonist (IL36RN), Caspase Recruitment Domain Family Member 14 (CARD14), Adaptor Related Protein Complex 1 Subunit Sigma 3 (AP1S3), Myeloperoxidase (MPO) and Serpin Peptidase Inhibitor Clade A Member 3 (SERPINA3) genes. Treatment guidelines for GPP are not well-entrenched. Currently, only one GPP-specific treatment, the interleukin-36 receptor antagonist (IL-36Ra) spesolimab, has been approved for use in the United States. Additional anti-IL-36 pathway therapies are currently being developed. Other treatment options include other biologic therapies such as IL-17 inhibitors, IL-23 inhibitors and TNFα inhibitors. Non-biologic therapeutic options include retinoids, cyclosporine and methotrexate. Treatment options differ throughout the world; most countries utilize retinoids, cyclosporine and methotrexate as first-line non-biologic options. China and United Kingdom have no GPP-specific biologic therapies approved for use, while several biologic therapies are approved for use in Japan. This review aims to serve as an update on the current global management of GPP while also including relevant aspects of disease pathogenesis, diagnosis, clinical presentation, histopathology, aetiology and epidemiology.