Clinical characteristics, disease burden, and unmet medical needs of generalized pustular psoriasis: Results of the SCRIPTOR study

IF 2.8 4区医学 Q2 DERMATOLOGY

Annales De Dermatologie Et De Venereologie Pub Date : 2025-09-02 DOI:10.1016/j.annder.2025.103427

F. Aubin , E. Wierzbicka-Hainaut , M. Vidal , M. Najean , E. Begon

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引用次数: 0

Abstract

Background

Generalized pustular psoriasis (GPP) is a rare, severe, chronic neutrophilic skin disease involving the interleukin-36 (IL-36) pathway.

Objective

The main objective of the SCRIPTOR international non-interventional study was to describe sociodemographic and clinical characteristics of GPP. This paper focuses on data collected from participating French centers.

Methods

Patients with a confirmed diagnosis of GPP between 2011 and 2022 were selected by dermatologists from 21 French hospitals. The clinical parameters evaluated included cutaneous and extracutaneous symptoms, and GPP flares in terms of number, duration, and impact. Secondary objectives included the description of treatment patterns.

Results

The analysis included 103 patients. The median age at diagnosis was 59 years. Median follow-up duration was 5 (1 to 11) years. Plaque psoriasis was reported in 48 % of patients. The most frequent comorbidities were hypertension (43 %), diabetes (25 %), dyslipidemia (17 %) and obesity (14 %). Forty-one patients (40 %) experienced flares. The median number of flares per patient was 2 and the median (min–max) duration of episodes was 19 (0–206) days. Treatments mainly included topical steroids and retinoids. Biological therapies were used in less than 10 % of patients at baseline. One death due to septic shock was related to GPP.

Conclusion

This is the largest French chart review study of patients with a confirmed diagnosis of GPP. The nature and prevalence of comorbidities, the length of flare episodes and the number of hospitalizations illustrate the burden of GPP. This study confirms that the impact of GPP should not be underestimated and supports the important need for targeted therapies.

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全身性脓疱性银屑病的临床特征、疾病负担和未满足的医疗需求：SCRIPTOR研究的结果

背景：广泛性脓疱性牛皮癣（GPP）是一种罕见的、严重的、慢性中性粒细胞性皮肤病，涉及白细胞介素-36 （IL-36）通路。目的：SCRIPTOR国际非介入研究的主要目的是描述GPP的社会人口学和临床特征。本文着重于从参与的法国中心收集的数据。方法由法国21家医院皮肤科医生选择2011 - 2022年确诊为GPP的患者。评估的临床参数包括皮肤和皮外症状，以及GPP耀斑的数量、持续时间和影响。次要目的包括对治疗模式的描述。结果纳入103例患者。诊断时的中位年龄为59 岁。中位随访时间为5年（1 ~ 11年）。斑块型银屑病在48 %的患者中被报道。最常见的合并症是高血压（43% %）、糖尿病（25% %）、血脂异常（17% %）和肥胖（14% %）。41例患者（40. %）出现耀斑。每位患者的中位数耀斑次数为2次，发作的中位数（最小-最大）持续时间为19（0-206）天。治疗主要包括局部类固醇和类维生素a。在基线时，使用生物疗法的患者不到10% %。1例脓毒性休克死亡与GPP有关。结论：这是法国对确诊为GPP的患者进行的最大的回顾性研究。合并症的性质和流行程度、发作时间和住院次数说明了GPP的负担。这项研究证实，GPP的影响不应被低估，并支持对靶向治疗的重要需求。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Annales De Dermatologie Et De Venereologie 医学-皮肤病学

CiteScore

1.40

自引率

0.00%

发文量

审稿时长

81 days

期刊介绍： Les Annales de dermatologie sont le rendez-vous mensuel incontournable de toute la dermatologie francophone, grâce à leur comité de rédaction qui assure une sélection rigoureuse des articles selon les normes de l''édition scientifique internationale. Une revue didactique, véritable aide à la pratique médicale quotidienne Pour compléter et enrichir la partie scientifique, la rubrique Formation médicale continue propose aux lecteurs des textes didactiques et interactifs (Cas pour diagnostic, Notes de pharmacovigilance, la Question du praticien, Dermatologie chirurgicale, la Sélection bibliographique du mois...) qui les font bénéficier d''une formation post-universitaire diversifiée et de qualité. La revue consacre également un espace pour la publication de questions des lecteurs auxquelles des experts apportent une réponse.