Bhutani Tina, Farberg Aaron S
Psoriasis and Skin Treatment Center, University of California at San Francisco, San Francisco, CA, 94118, USA.
Bare Dermatology, Dallas, TX, USA.
Dermatol Ther (Heidelb). 2024 Feb;14(2):341-360. doi: 10.1007/s13555-024-01103-5. Epub 2024 Feb 16.
Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening disease. There is limited understanding of patient characteristics in GPP and their correlation with disease progression or healthcare resource utilization. Our review aims to examine real-world evidence on these characteristics and the associated disease burden as related to economic and quality of life factors. Results showed that most patients with GPP experienced flares once a year, lasting from 2 weeks to 3 months, with > 80% of patients having residual disease post-flare, with/without treatment, indicating the long-term nature of GPP. The impact of GPP on patients' daily activities was significant, even in the absence of a flare. GPP adversely affected mental health, and anxiety and depression were reported regularly. Patients with GPP had more comorbidities, were prescribed more medication, and had more inpatient and outpatient visits than in matched plaque psoriasis or general population cohorts. Improving the education of healthcare providers in diagnosing GPP, defining disease flares, and managing the disease, as well as making globally accepted clinical guidelines for GPP treatment available, could help to reduce the burden on patients with GPP. Effective therapies that control and prevent GPP flares and manage chronic disease are needed.
泛发性脓疱型银屑病(GPP)是一种慢性、罕见且可能危及生命的疾病。目前对GPP患者特征及其与疾病进展或医疗资源利用之间的相关性了解有限。我们的综述旨在研究关于这些特征以及与经济和生活质量因素相关的疾病负担的真实世界证据。结果显示,大多数GPP患者每年发作一次,持续2周 至3个月,超过80%的患者在发作后无论是否接受治疗都有疾病残留,这表明GPP具有长期性。即使在无发作期间,GPP对患者日常活动的影响也很显著。GPP对心理健康有不利影响,焦虑和抑郁的报告很常见。与匹配的斑块状银屑病或普通人群队列相比,GPP患者有更多的合并症,服用更多的药物,住院和门诊就诊次数也更多。提高医疗服务提供者对GPP的诊断、疾病发作的定义和疾病管理的认识,以及制定全球公认的GPP治疗临床指南,有助于减轻GPP患者的负担。需要有效的疗法来控制和预防GPP发作并管理慢性病。