Clinical characteristics of Japanese pustular psoriasis: A multicenter observational study
Clinical characteristics of Japanese pustular psoriasis: A multicenter observational study
复制标题
DOI：
10.1111/1346-8138.16217
复制
发表时间：
2021-11-01
影响因子：
3.1
通讯作者：
Imafuku, Shinichi
中科院分区：
文献类型：
--
作者：
Ohata, Chika;Tsuruta, Noriko;Imafuku, Shinichi
泛发性脓疱型银屑病（GPP）是银屑病的一种罕见而严重的亚型。由于其罕见性，大样本量的GPP研究很少。我们使用截至2020年12月底登记的西日本银屑病登记处的数据研究了GPP和脓疱性银屑病的特征。该数据集包括104例脓疱型银屑病患者和1290例其他亚型银屑病患者。多变量分析显示，与其他银屑病亚型相比，脓疱型银屑病女性患者数量显著增加，平均体重指数显著降低，习惯性饮酒者比例显著降低。在104名患者中，102名患有GPP，包括88名von Zumbusch，10名青少年发病型银屑病和4名环状脓疱型银屑病。虽然男性：GPP合并寻常型银屑病（GPP+PsV）的女性比例（47/20）与日本银屑病相似，GPP不伴PsV（GPP-PsV）组以女性为主（13/22）。GPP发作时的平均年龄为45.3岁，从PsV发作到GPP发作的平均间隔为12.5年。9例GPP患者中有4例存在IL 36 RN基因突变。感染、药物、妊娠是GPP的诱发因素。8例（7.8%）GPP患者有银屑病家族史。24例GPP患者有银屑病关节炎。76.5%的GPP患者使用生物制剂，其次是依维甲酸酯（37.3%）、环孢素（24.5%）、甲氨蝶呤（13.7%）、阿普斯特（8.8%）以及粒细胞和单核细胞吸附单采术（6.9%）。在33例GPP患者中，17例（51.5%）使用了Eretinate，病史小于10年。因此，即使在生物制剂时代，etretinate仍然是GPP的良好治疗选择。高血压是最常见的合并症，其次是糖尿病。我们相信，在这项研究中揭示的特征可以进一步有助于有效的GPP管理。
Generalized pustular psoriasis (GPP) is a rare and severe subtype of psoriasis. Because of its rarity, GPP studies with a large sample size have been scarce. We studied the characteristics of GPP and pustular psoriasis using data from the West Japan Psoriasis Registry that had been registered until the end of December 2020. The dataset included 104 patients with pustular psoriasis and 1290 patients with other subtypes of psoriasis. Multivariate analysis revealed a significantly greater number of female patients, a significantly lower mean body mass index, and a significantly lower ratio of habitual drinkers in pustular psoriasis, compared to other subtypes of psoriasis. Of the 104 patients, 102 had GPP, including 88 von Zumbusch, 10 juvenile-onset, and four annular pustular psoriasis. Although the male : female ratio of GPP with psoriasis vulgaris (GPP+PsV) (47/20) was similar to that of psoriasis in Japan, the GPP without PsV (GPP-PsV) group highlighted a female predominance (13/22). The mean age at GPP onset was 45.3 years, and the mean interval from PsV onset to GPP onset was 12.5 years. Four of nine patients with GPP had an IL36RN gene mutation. Infection, medicine, and pregnancy were the precipitating factors for GPP. A family history of psoriasis was present in eight (7.8%) patients with GPP. Twenty-four patients with GPP had psoriatic arthritis. Biologics were used in 76.5% of patients with GPP, followed by etretinate (37.3%), cyclosporine (24.5%), methotrexate (13.7%), apremilast (8.8%), and granulocyte and monocyte adsorption apheresis (6.9%). Etretinate was used in 17 (51.5%) of 33 patients with GPP with less than 10-year history. Thus, etretinate remains a good treatment option for GPP even in the era of biologics. Hypertension was the most commonly identified comorbidity, followed by diabetes. We believe that the characteristics revealed in this study can further contribute to effective GPP management.