Rega Federica, Trovato Federica, Bortone Giulio, Pellacani Giovanni, Richetta Antonio Giovanni, Dattola Annunziata
Dermatology Unit, Department of Clinical Internal, Anesthesiological and Cardiovascular Science, University of La Sapienza, Rome, Italy.
Psoriasis (Auckl). 2024 Mar 15;14:23-27. doi: 10.2147/PTT.S393978. eCollection 2024.
Generalized pustular psoriasis (GPP) is a rare, chronic, and severe skin disorder characterized by the eruption of non-infectious pustules on an erythematous background often associated with systemic symptoms. It may appear in association with plaque psoriasis or occur in previously healthy individuals. It differs from psoriasis vulgaris in clinical presentation, immunopathogenesis, histology, and therapeutic strategies. Overexpression of interleukin 36 (IL-36) or a loss-of-function mutation of IL-36 receptor antagonist (IL-36RA) are thought to play a pivotal role in the pathogenesis of this disease. There are currently no globally approved guidelines for the treatment of GPP, and the therapies used so far, with variable results, have given unsatisfactory results. Spesolimab, a selective humanized antibody against the IL-36 receptor that blocks its activation, is the first biologic drug approved in Europe in December 2022 for the treatment of GPP flares. It represents a promising therapy, demonstrating efficacy in reducing disease severity and improving patient outcomes. In our review, we have analyzed the latest advancements and findings regarding the efficacy and safety of spesolimab in the context of GPP management.
泛发性脓疱型银屑病(GPP)是一种罕见的、慢性的、严重的皮肤疾病,其特征是在红斑背景上出现非感染性脓疱,常伴有全身症状。它可能与斑块状银屑病同时出现,也可能发生在既往健康的个体中。其临床表现、免疫发病机制、组织学和治疗策略均与寻常型银屑病不同。白细胞介素36(IL-36)的过度表达或IL-36受体拮抗剂(IL-36RA)的功能丧失突变被认为在该疾病的发病机制中起关键作用。目前尚无全球公认的GPP治疗指南,迄今为止所使用的治疗方法效果不一,均未取得令人满意的结果。司库奇尤单抗是一种针对IL-36受体的选择性人源化抗体,可阻断其激活,是2022年12月在欧洲获批用于治疗GPP发作的首个生物药物。它是一种有前景的治疗方法,在降低疾病严重程度和改善患者预后方面显示出疗效。在我们的综述中,我们分析了司库奇尤单抗在GPP管理背景下的疗效和安全性方面的最新进展和研究结果。