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Diagnosis Screening and Treatment of Patients with Palmoplantar Pustulosis (PPP): A Review of Current Practices and Recommendations

机译:伴有棕榈术毛囊患者(PPP)的诊断筛选和治疗:对现有实践和建议的审查

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摘要

Palmoplantar pustulosis (PPP) is a rare, chronic, recurrent inflammatory disease that affects the palms and/or the soles with sterile, erupting pustules, which are debilitating and usually resistant to treatment. It has genetic, histopathologic and clinical features that are not present in psoriasis; thus, it can be classified as a variant of psoriasis or as a separate entity. Smoking and upper respiratory infections have been suggested as main triggers of PPP. PPP is a challenging disease to manage, and the treatment approach involves both topical and systemic therapies, as well as phototherapy and targeted molecules. No gold standard therapy has yet been identified, and none of the treatments are curative. In patients with mild disease, control may be achieved with on-demand occlusion of topical agents. In patients with moderate-to-severe PPP, phototherapy or a classical systemic agent (acitretin being the best treatment option, especially in combination with PUVA) may be effective. Refractory patients or those with contraindications to use these therapies may be good candidates for apremilast or biologic therapy, particularly anti-IL-17A and anti-IL-23 agents. Recent PPP trials are focusing on blockage of IL-36 or IL-1 pathways, which play an important role in innate immunity. Indeed, IL-36 isoforms have been strongly implicated in the pathogenesis of psoriasis. Therefore, blockage of the IL-36 pathway has become a new treatment target in PPP, and three studies are currently evaluating the use of monoclonal antibodies that block the IL-36 receptor in PPP: ANB019 and spesolimab (BI 655130). In this review, we explore the diagnosis, screening and treatment of patients with PPP.
机译:棕榈叶腭裂(PPP)是一种罕见的慢性,炎症性炎症疾病,其影响棕榈树和/或具有无菌喷发脓疱的鞋底,这些缺陷症是衰弱的,并且通常是抗性的。它具有不存在于牛皮癣中的遗传,组织病理学和临床特征;因此,它可以被分类为牛皮癣的变体或作为单独的实体。已经提出了吸烟和上呼吸道感染作为PPP的主要触发。 PPP是一种挑战性的疾病,治疗方法涉及局部和系统疗法,以及光疗和靶向分子。尚未确定黄金标准疗法,并且没有任何治疗方法是治愈性的。在轻度疾病的患者中,可以通过按需闭塞来实现对照的局部剂。在中度至严重的PPP患者中,光疗或经典的全身性能(丙酸是最佳治疗选项,特别是与PUVA组合)可能是有效的。难治性患者或具有禁忌症的患者这些疗法可能是用于食用的食物或生物治疗,特别是抗IL-17A和抗IL-23代理的良好候选者。最近的PPP试验专注于IL-36或IL-1途径的障碍,这在先天免疫中起重要作用。实际上,IL-36同种型已经强烈地涉及牛皮癣的发病机制。因此,IL-36途径的阻塞已成为PPP中的新治疗靶标,目前有三项研究正在评估嵌段在PPP:ANB019和Spsolimab中的IL-36受体的单克隆抗体(BI 655130)。在本综述中,我们探讨了PPP患者的诊断,筛选和治疗。

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