Patient Experiences on the Diagnosis, Management, and Burden of Generalized Pustular Psoriasis: An International Web Survey and Qualitative Interview Study

泛发性脓疱型银屑病患者的诊断、治疗和负担体验:一项国际网络调查和定性访谈研究

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Abstract

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare, serious, chronic, neutrophilic skin disease, distinct from plaque psoriasis, characterized by recurrent flares of cutaneous erythema and widespread sterile pustules. Patient-centric data on the quality of life (QoL) impacts of GPP remain limited. OBJECTIVE: To evaluate the physical and emotional QoL impacts of GPP. METHODS: We used a web-based survey and 90-minute telephone interviews with open-ended questions to evaluate the experiences of adults with GPP across China, Japan, the UK, and the USA. Quantitative measures included the Dermatology Life Quality Index (DLQI), Psoriasis Symptom Scale (PSS), and Worst GPP Pain Numeric Rating Scale (NRS). RESULTS: 21 participants completed the survey; 9 completed telephone interviews. The journey to diagnosis was complicated, with 48% of participants seeing ≥4 physicians before the diagnosis. Impacts of GPP on participants physical and mental QoL were substantial, such as the anxiety of not being able to plan life and work with confidence, both in general and during flares. The mean DLQI was 10.2, and the PSS and Worst GPP Pain NRS were highest in participants experiencing recent flares. Participants' biggest worries included risk of flares, distress from symptoms, inadequate treatment, and passing on the disease to their children. Limitations included a small sample size and short recall timeframe. CONCLUSION: Participants reported multiple impacts of GPP on their daily lives even in the absence of acute flares, highlighting the need for greater disease awareness and understanding among healthcare professionals.

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