Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008, Warsaw, Poland.
Am J Clin Dermatol. 2020 Jun;21(3):355-370. doi: 10.1007/s40257-020-00503-5.
Palmoplantar pustulosis (PPP) is a chronic, recurrent skin disease belonging to the spectrum of psoriasis. It is characterized by an eruption of sterile pustules on the palms and soles. Recent studies in PPP have focused on genetic differences between pustular phenotypes and the role of the innate immunological system and the microbiome in the etiopathogenesis of the disease. Mutations in IL36RN (a major predisposing factor for generalized pustular psoriasis) were found in selected patients with PPP and were associated with earlier disease onset. Studies have shown that the interleukin (IL)-17 and IL-36 pathways might be involved in the pathogenesis of PPP. A microbiome has been demonstrated in the vesicopustules of PPP, and an abundance of Staphylococcus appears to be increased by smoking. Improved understanding of the underlying etiopathogenesis of PPP has led to advances in treatment options, and targeted therapies for PPP have been evaluated or are under evaluation against more than 12 molecules in ongoing clinical trials. These targets include CXCR2 (IL-8 receptor type B), granulocyte colony-stimulating factor receptor, IL-1 receptor, IL-8, IL-12, IL-23, IL-17A, IL-17 receptor, IL-36 receptor, phosphodiesterase-4, and tumor necrosis factor-α.
掌跖脓疱病 (PPP) 是一种慢性、复发性皮肤病，属于银屑病谱。其特征是手掌和脚底出现无菌脓疱。近期 PPP 的研究集中在脓疱表型之间的遗传差异以及固有免疫系统和微生物组在疾病发病机制中的作用。在选定的 PPP 患者中发现了 IL36RN（泛发性脓疱性银屑病的主要易感因素）突变，与疾病更早发作相关。研究表明，白细胞介素 (IL)-17 和 IL-36 通路可能参与 PPP 的发病机制。PPP 的水疱脓疱中存在微生物组，而吸烟似乎会增加金黄色葡萄球菌的丰度。对 PPP 潜在发病机制的深入了解导致治疗选择的进步，针对超过 12 种分子的靶向治疗已在进行中的临床试验中进行了评估或正在评估。这些靶点包括 CXCR2（IL-8 受体类型 B）、粒细胞集落刺激因子受体、IL-1 受体、IL-8、IL-12、IL-23、IL-17A、IL-17 受体、IL-36 受体、磷酸二酯酶-4 和肿瘤坏死因子-α。
