全身性疱疹性银屑病

定义

泛发性脓疱性银屑病是一种严重的炎症性皮肤病，可危及生命，其特征是反复发作高热、疲劳、偶发性红斑性皮肤疹，身体各部位出现无菌性脓疱，以及中性粒细胞增多症。

别名

GPP

基本事实

遗传方式

常染色体隐性、不适用

发病年龄

成年期

患病率

1-9 / 1 000 000（Europe）

相关基因 2

临床表型 27

近两年的全球研究 554L2

2024/08 起在 Europe PMC 检索所得，按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务，尚未引入。

• 2026-08综述
  INDIVIDUAL ARTICLE: Generalized Pustular Psoriasis: A Contemporary Review of Diagnosis, Pathophysiology, and IL-36 Pathway-Directed Management
  Journal of drugs in dermatology : JDD · DOI · Europe PMC
• 2026-07
  Treatment Patterns and Pathways of Systemic Drug Therapy in Patients With Plaque, Erythrodermic, and Generalized Pustular Psoriasis: A Retrospective Cohort Study in Japan
  The Journal of dermatology · DOI · Europe PMC
• 2026-06
  Deucravacitinib for Generalized Pustular Psoriasis and Comorbid Polymyositis: Dual Efficacy and Favorable Safety
  American journal of therapeutics · DOI · Europe PMC
• 2026-06病例报告
  Severe generalized pustular psoriasis complicated with staphylococcal toxic shock syndrome treated with spesolimab and antibiotics
  Journal der Deutschen Dermatologischen Gesellschaft = Journal of the G · DOI · Europe PMC
• 2026-06
  S1 Guideline: Therapy of generalized pustular psoriasis
  Journal der Deutschen Dermatologischen Gesellschaft = Journal of the G · DOI · Europe PMC
• 2026-06开放获取
  Dupilumab-induced generalized pustular psoriasis in an elderly patient: a case report and literature review
  Anais brasileiros de dermatologia · DOI · Europe PMC
• 2026-06系统综述综述
  Monotherapy with Biologics for Generalized Pustular Psoriasis: A Systematic Review of Comparative Interventional Studies with an Exploratory Network Meta-Analysis
  Medical sciences (Basel, Switzerland) · DOI · Europe PMC
• 2026-06开放获取
  Enhanced Diagnosis of Generalized Pustular Psoriasis With the Legit.Health Device as a Diagnosis Support Tool: Multireader Multicase Study
  JMIR dermatology · DOI · Europe PMC
• 2026-06开放获取
  Prior Specific Biologic Use as a Potential Risk Factor for Administration-related Reactions to Ixekizumab after Excipient Modification: A Single-centre Retrospective Study
  Acta dermato-venereologica · DOI · Europe PMC
• 2026-06开放获取
  Recurrent pustular psoriasis of pregnancy and postpartum flare
  CMAJ : Canadian Medical Association journal = journal de l'Association · DOI · Europe PMC
• 2026-05病例报告开放获取
  Secukinumab for lamellar ichthyosis in an adolescent: a case report
  Translational pediatrics · DOI · Europe PMC
• 2026-05开放获取
  Curcumin alleviates depression-aggravated psoriasis by targeting MMP9-regulated apoptotic pathways
  iScience · DOI · Europe PMC
• 2026-05综述
  Pustular Dermatoses. Part I. Inflammatory and Autoimmune
  Journal of the American Academy of Dermatology · DOI · Europe PMC
• 2026-05
  Generalized Pustular Psoriasis in Portugal: Expert Perspectives on Patient Journey, Diagnosis, Therapeutic Goals, and Healthcare Optimization
  Acta medica portuguesa · DOI · Europe PMC
• 2026-05综述开放获取
  The Role of Inflammatory Biomarkers in Disease Severity and Treatment Response across Psoriasis, Atopic Dermatitis, and Hidradenitis Suppurativa-A Narrative Review
  Dermatology and therapy · DOI · Europe PMC
• 2026-05综述开放获取
  Psoriasis in skin of color: clinical presentation, diagnostic challenges, and therapeutic considerations
  Anais brasileiros de dermatologia · DOI · Europe PMC
• 2026-05综述开放获取
  IL-36-Driven Inflammation in Generalized Pustular Psoriasis: Immunological Insights from Plaque Psoriasis and Implications for Targeted Therapy
  International journal of molecular sciences · DOI · Europe PMC
• 2026-05开放获取
  Sex-Specific Transcriptomic Profiles in Psoriatic Lesions: A Large-Scale Integrative Study
  International journal of molecular sciences · DOI · Europe PMC
• 2026-05开放获取
  Psoriasis in the Brazilian Public Health System: A Nationwide Analysis of Healthcare Utilization and Inequalities, 2014-2024
  Healthcare (Basel, Switzerland) · DOI · Europe PMC
• 2026-05
  Spesolimab for Generalized Pustular Psoriasis in the Late Second Trimester of Pregnancy
  The Journal of dermatology · DOI · Europe PMC

国家医保药品目录中点名本病的药品 1L2

出自《国家基本医疗保险、生育保险和工伤保险药品目录（2025年）》（医保发〔2025〕33号，2026-01-01 起执行）。下列药品在药品名称或限定支付范围里出现了本病的名称。

• 佩索利单抗注射液乙类谈判药品
  限成人泛发性脓疱型银屑病(GPP)。

境外已获批用于本病的药物 1L2

欧盟 0 项、美国 1 项。同一药物在两地各批一次的，会分别列出。

• Spevigo美国2022-09-01
  spesolimab-sbzo
  官方记录

数据来自欧洲药品管理局（EMA）的药品与孤儿药资格公开导出表，以及美国 FDA 孤儿药资格数据库。两边口径不同：欧盟一侧取的是当前状态仍为「已授权」的药品；美国一侧记录的是「曾获批准」这一事实，FDA 的公开表不追踪药物此后是否退市（例如 Relyvrio 于 2024 年撤市，表中仍记为已获批）。请以官方记录页为准。

在中国开展的临床试验 17L2

按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实，并与主治医生商量。

登记为可入组 7

• 招募中NCT06231381
  Efficacy and Safety of HB0034 in Patients with Generalized Pustular Psoriasis (GPP)
  II 期 · 干预性 · 2024/02/29Shanghai Huaota Biopharmaceutical Co., Ltd.
  中国研究中心 2 个：Beijing、Shanghai
• 招募中NCT06477536
  Long-Term Safety and Efficacy of HB0034 in Subjects With Generalized Pustular Psoriasis
  II 期、III 期 · 干预性 · 2024/09/02Shanghai Huaota Biopharmaceutical Co., Ltd.
  中国研究中心 3 个：Beijing、Hangzhou
• 招募中NCT07270003
  A Prospective, Single-arm, Open-label Clinical Trial to Evaluate the Efficacy and Safety of Ivarmacitinib in the Treatment of Palmoplantar Pustulosis
  不适用 · 干预性 · 2025/10/01Xijing Hospital
  中国研究中心 1 个：Xi'an
• 招募中NCT07219420
  A Study to Evaluate the Efficacy and Safety of Bimekizumab in Study Participants With Palmoplantar Pustulosis
  III 期 · 干预性 · 2025/11/14UCB Biopharma SRL
  中国研究中心 15 个：Beijing、Chengdu、Chongqing、Guangzhou、Hangzhou、Jinan 等 11 地
• 招募中NCT07503652
  Clinical Observation of Xeligekimab in the Treatment of Moderate to Severe Palmoplantar Pustulosis
  IV 期 · 干预性 · 2026/03First Hospital of China Medical University
  中国研究中心 1 个：Shenyang
• 招募中NCT07552454
  Picankibart in Palmoplantar Pustulosis
  不适用 · 干预性 · 2026/04/15Xijing Hospital
  中国研究中心 1 个：Xi'an
• 尚未开始招募NCT07530367
  Tofacitinib Plus Imatinib in Moderate-to-Severe Palmoplantar Pustulosis
  III 期 · 干预性 · 2026/08/01Second Affiliated Hospital, Zhejiang University, School of Medicine
  中国研究中心 1 个：Hangzhou

中国境外的在招试验 13L2

CT.gov 报告命中 13 项，此处取回并展示最近的 6 项。

• 尚未开始招募NCT07448428
  Generalized Pustular Psoriasis Registry in Costa Rica
  观察性 · 2026/05Caja Costarricense de Seguro Social
  Costa Rica
• 招募中NCT07013201
  A 16-week Trial to Investigate the Efficacy and Safety of Delgocitinib Cream 20 mg/g in Adult Participants With Mild to Severe Palmoplantar Pustulosis
  II 期 · 干预性 · 2025/09/11LEO Pharma
  加拿大、德国、波兰、英国、美国
• 尚未开始招募NCT06790940
  Construction of a Psoriasis and Psoriatic Arthritis Diagnostic Model Based on Multidimensional Nail Information
  观察性 · 2025/01/20Shanghai Yueyang Integrated Medicine Hospital
• 招募中NCT05994976
  Collection of Samples From Subjects With Various Skin Conditions and Healthy Volunteers
  观察性 · 2023/10/17Innovaderm Research Inc.
  加拿大
• 招募中NCT06100991
  CorEvitas Generalized Pustular Psoriasis (GPP) Drug Safety and Effectiveness Registry
  观察性 · 2023/09/19CorEvitas
  美国
• 招募中NCT04359394
  International Rare And Severe Psoriasis Expert Network
  观察性 · 2021/09/22University Hospital, Basel, Switzerland
  德国、意大利、葡萄牙、新加坡、瑞士、土耳其

外部标识与链接