泛发性脓疱型银屑病的现状：基于127例中国患者的多中心医院调查结果
Current status of generalized pustular psoriasis: Findings from a multicenter hospital-based survey of 127 Chinese patients.
作者信息
Wang Haimeng, Xu Jiaming, Yu Xiaoling, Hao Siyu, Chen Xueqin, Peng Bin, Li Xiaona, Wang Ping, Miao Chaoyang, Guo Jinzhu, Hu Qingjie, Su Zhonglan, Wang Sheng, Yu Chen, Sun Qingmiao, Zhang Minkuo, Yang Bin, Li Yuzhen, Song Zhiqiang, Geng Songmei, Chen Aijun, Xu Zigang, Zhang Chunlei, Lu Qianjin, Lu Yan, Jiang Xian, Wang Gang, Fang Hong, Sun Qing, Liu Jie, Jin Hongzhong
机构信息
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing 100730, China.
Department of Dermatology, Dermatology Hospital of Southern Medical University, Guangzhou, Guangdong 510091, China.
出版信息
Chin Med J (Engl). 2025 Apr 20;138(8):953-961. doi: 10.1097/CM9.0000000000003494. Epub 2025 Mar 17.
BACKGROUND
Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited.
METHODS
This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed.
RESULTS
Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]-3rd quartile [Q3]: 11-44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset ( P = 0.021) and transitioning to plaque psoriasis ( P = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1-Q3: 2-41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1-Q3: 13.0-23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP.
CONCLUSIONS
The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.
背景
泛发性脓疱型银屑病（GPP）是一种罕见的复发性自身炎症性疾病，给患者和社会带来了沉重负担。中国对GPP的认知仍然有限。
方法
这项横断面调查于2021年9月至2023年5月在中国的14家医院开展，纳入了所有年龄和疾病阶段的GPP患者。收集的数据包括人口统计学信息、临床特征、经济影响、疾病严重程度、生活质量以及治疗相关并发症。分析了GPP复发的危险因素。
结果
在127例患者中（女性/男性比例 = 1.35:1），疾病发病的平均年龄为25岁（第一四分位数[Q1] - 第三四分位数[Q3]：11 - 44岁）；29.2%的患者患GPP超过10年。75.6%的患者出现复发，近一半患者表示没有可识别的诱发因素。发病年龄较小（P = 0.021）和转变为斑块状银屑病（P = 0.022）与较高的复发率相关。诊断延迟的中位数为8个月（Q1 - Q3：2 - 41个月），32.3%的患者报告有误诊情况。53.5%的患者存在合并症，而51.1%的患者在治疗期间出现全身并发症。抑郁和焦虑分别影响了84.
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