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Abstract：
BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and chronic, debilitating skin condition characterized, in its acute flare phase, by clinically severe and potentially life-threatening systemic manifestations. Data on GPP are still scanty, particularly in Europe and at a national level. The aim of this study was to provide expert indications on several disease-related and patient-related aspects of GPP, with specific focus to the Italian context.
METHODS: We conducted an iterative eDelphi study following the recommended criteria for reporting methods and results. After a thorough bibliographic review aimed to identify unknown or controversial issues in GPP, the following areas were investigated through a few specific questions/statements for each area: (1) disease epidemiology; (2) disease characteristics, with specific interest toward GPP flares; (3) diagnosis and diagnostic delay; (4) GPP treatment; (5) GPP patient journey and use of healthcare resources in Italy; (6) unmet needs and quality of life. An Executive Board of 9 principal investigators revised and approved the topics to be examined and overviewed the whole project. A total of 35 experts from different Italian areas, including 34 board-certified Italian dermatologists and 1 representative of patients\' associations, took part in the study.
RESULTS: A high agreement in responses from Italian experts emerged during two eDelphi iterations on - among several other aspects - GPP prevalence and incidence in Italy, use of European Rare and Severe Psoriasis Expert Network diagnostic criteria, flare frequency and duration, best diagnostic and care pathway, and main unmet needs of Italian patients. On the other hand, a broad spectrum of treatments (of different drug classes) was reported both in the acute and chronic phases of GPP, and no consensus on the issue was thus achieved.
CONCLUSIONS: Consensus findings from this Delphi study of GPP experts may be useful to fill gaps of knowledge and improve awareness of this rare disease, as well as to help clinical and public health management of GPP in Italy.
METHODS: We conducted an iterative eDelphi study following the recommended criteria for reporting methods and results. After a thorough bibliographic review aimed to identify unknown or controversial issues in GPP, the following areas were investigated through a few specific questions/statements for each area: (1) disease epidemiology; (2) disease characteristics, with specific interest toward GPP flares; (3) diagnosis and diagnostic delay; (4) GPP treatment; (5) GPP patient journey and use of healthcare resources in Italy; (6) unmet needs and quality of life. An Executive Board of 9 principal investigators revised and approved the topics to be examined and overviewed the whole project. A total of 35 experts from different Italian areas, including 34 board-certified Italian dermatologists and 1 representative of patients\' associations, took part in the study.
RESULTS: A high agreement in responses from Italian experts emerged during two eDelphi iterations on - among several other aspects - GPP prevalence and incidence in Italy, use of European Rare and Severe Psoriasis Expert Network diagnostic criteria, flare frequency and duration, best diagnostic and care pathway, and main unmet needs of Italian patients. On the other hand, a broad spectrum of treatments (of different drug classes) was reported both in the acute and chronic phases of GPP, and no consensus on the issue was thus achieved.
CONCLUSIONS: Consensus findings from this Delphi study of GPP experts may be useful to fill gaps of knowledge and improve awareness of this rare disease, as well as to help clinical and public health management of GPP in Italy.
摘要：
背景：泛发性脓疱型银屑病（3GPP）是一种罕见且慢性的，衰弱的皮肤状况特征，在急性耀斑阶段，临床严重和潜在危及生命的全身表现。3GPP上的数据仍然很少，特别是在欧洲和国家层面。这项研究的目的是提供有关的几个疾病相关和患者相关方面的专家适应症。特别关注意大利背景。
方法：我们按照报告方法和结果的推荐标准进行了迭代的eDelphi研究。经过彻底的书目审查，目的是确定阵未知或有争议的问题，通过对每个领域的一些具体问题/陈述，调查了以下领域:(1)疾病流行病学；(2)疾病特征，特别感兴趣的是对3GPP耀斑；（3）诊断和诊断延迟；（4）3GPP治疗；（5）意大利的3GPP患者旅程和医疗保健资源的使用；（6）未满足的需求和生活质量。由9名主要调查人员组成的执行委员会修订并批准了要审查的主题，并概述了整个项目。共有35位来自意大利不同地区的专家，包括34名经委员会认证的意大利皮肤科医生和1名患者协会代表，参加了这项研究。
结果：在两次eDelphi迭代中，意大利专家的回答达成了高度一致-除其他几个方面外-意大利的3GPP患病率和发病率，使用欧洲罕见和严重银屑病专家网络诊断标准，耀斑频率和持续时间，最好的诊断和护理途径，以及意大利患者的主要未满足需求。另一方面,广谱的治疗（不同的药物类别）被报道在急性和慢性期的GMP，因此，在这个问题上没有达成共识。
结论：从这个德尔菲研究的3GPP专家的共识发现可能有助于填补知识空白和提高对这种罕见疾病的认识，以及帮助意大利的临床和公共卫生管理。
方法：我们按照报告方法和结果的推荐标准进行了迭代的eDelphi研究。经过彻底的书目审查，目的是确定阵未知或有争议的问题，通过对每个领域的一些具体问题/陈述，调查了以下领域:(1)疾病流行病学；(2)疾病特征，特别感兴趣的是对3GPP耀斑；（3）诊断和诊断延迟；（4）3GPP治疗；（5）意大利的3GPP患者旅程和医疗保健资源的使用；（6）未满足的需求和生活质量。由9名主要调查人员组成的执行委员会修订并批准了要审查的主题，并概述了整个项目。共有35位来自意大利不同地区的专家，包括34名经委员会认证的意大利皮肤科医生和1名患者协会代表，参加了这项研究。
结果：在两次eDelphi迭代中，意大利专家的回答达成了高度一致-除其他几个方面外-意大利的3GPP患病率和发病率，使用欧洲罕见和严重银屑病专家网络诊断标准，耀斑频率和持续时间，最好的诊断和护理途径，以及意大利患者的主要未满足需求。另一方面,广谱的治疗（不同的药物类别）被报道在急性和慢性期的GMP，因此，在这个问题上没有达成共识。
结论：从这个德尔菲研究的3GPP专家的共识发现可能有助于填补知识空白和提高对这种罕见疾病的认识，以及帮助意大利的临床和公共卫生管理。
