Megna Matteo, Camela Elisa, Ruggiero Angelo, Battista Teresa, Martora Fabrizio, Cacciapuoti Sara, Potestio Luca
Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.
Dermatology Unit, Istituto Dermopatico dell'Immacolata - IRCCS, Rome, Italy.
Clin Cosmet Investig Dermatol. 2023 Jun 28;16:1677-1690. doi: 10.2147/CCID.S407812. eCollection 2023.
Generalized pustular psoriasis (GPP) is a severe and rare form of psoriasis, being a potentially life-threatening condition, characterized by recurring episodes or flares of widespread cutaneous erythema with macroscopic sterile pustules. An irregular innate immune response is linked to GPP, which is considered an auto-inflammatory disorder, while innate and adaptive immunopathogenic responses are involved in psoriasis pathogenesis. In consequence, different cytokine cascades have been suggested to be mainly involved in the pathogenesis of each different psoriasis form, with the interleukin (IL)23/IL17 axis implied in plaque psoriasis, and the IL36 pathway in the GPP. As regards GPP treatment, conventional systemic drugs available for plaque psoriasis are usually used as the first-line treatment option. However, contraindications and adverse events often limit the use of these therapies. In this scenario, biologic drugs may represent a promising treatment option. To date, even if 12 different biologics have been approved for plaque psoriasis, none of these is approved for GPP where they are employed off-label. Recently, spesolimab, an anti-IL36 receptor monoclonal antibody, has been recently approved for GPP. The purpose of this article is to assess the current literature about the use of biological therapies for the treatment of GPP to establish the basis for a shared GPP management algorithm.
泛发性脓疱型银屑病（GPP）是一种严重且罕见的银屑病形式，是一种潜在的危及生命的疾病，其特征为反复出现或发作的广泛皮肤红斑伴肉眼可见的无菌脓疱。不规则的先天免疫反应与GPP相关，GPP被认为是一种自身炎症性疾病，而先天和适应性免疫致病反应参与银屑病的发病机制。因此，不同的细胞因子级联反应被认为主要参与每种不同银屑病形式的发病机制，白介素（IL）23/IL17轴与斑块状银屑病有关，而IL36途径与GPP有关。关于GPP的治疗，可用于斑块状银屑病的传统全身性药物通常用作一线治疗选择。然而，禁忌证和不良事件常常限制了这些疗法的使用。在这种情况下，生物药物可能是一种有前景的治疗选择。迄今为止，即使有12种不同的生物制剂已被批准用于斑块状银屑病，但这些生物制剂均未被批准用于GPP，目前在GPP中的使用属于超说明书用药。最近，抗IL36受体单克隆抗体司库奇尤单抗最近已被批准用于GPP。本文的目的是评估有关使用生物疗法治疗GPP的现有文献，为共享的GPP管理算法奠定基础。
