Xin Qian Seah, Siew Chin Ong, Mustapha Mohammed, Mei Ee Tay, Latha Selvarajah, Wooi Chiang Tan, Sook Yee Michelle Voo, Yen Loo Ting, Jyh Jong Tang, Azura Mohd Affandi
{"title":"马来西亚成人广泛性脓疱性牛皮癣患者的健康相关生活质量:一项横断面研究","authors":"Xin Qian Seah, Siew Chin Ong, Mustapha Mohammed, Mei Ee Tay, Latha Selvarajah, Wooi Chiang Tan, Sook Yee Michelle Voo, Yen Loo Ting, Jyh Jong Tang, Azura Mohd Affandi","doi":"10.1186/s13023-025-03820-2","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Generalized Pustular Psoriasis (GPP) is a rare but severe form of psoriasis, characterized by flares involving the sudden spread of erythema with sterile pustules, crusting, and scaling.</p><p><strong>Objective: </strong>The study aimed to assess both generic and dermatology-specific health-related quality of life (HRQoL) in patients with GPP across flare and non-flare stages in Malaysia.</p><p><strong>Methods: </strong>The study was a multicenter cross-sectional study conducted among patients diagnosed with GPP attending the General Hospitals under the Ministry of Health, Malaysia, between May 15, 2024, and November 30, 2024. The HRQoL of the GPP patients were assessed using the validated versions of the Dermatology Life Quality Index (DLQI) and EuroQol-5 Dimensions (EQ-5D). Data were statistically analysed using descriptive and inferential statistics using IBM SPSS version 28.0.</p><p><strong>Results: </strong>A total of fifty-four patients from six centres were enrolled into the study, with a mean (standard deviation, SD) age of 44.6 (SD: 15.5), the majority being female (n = 41, 75.9%). The mean (SD) scores of the DLQI and EQ-5D for the patients with GPP were 8.44 (SD: 7.12) and 0.74 (SD: 0.27), respectively. Thirteen patients (24%) were in their flares phase at the time of assessment. During the flares stage, patients experienced poorer quality of life, as indicated by a significantly higher mean DLQI score of 15.70 (SD: 7.33) (p < 0.001), lower EQ-5D score of 0.41 (SD: 0.30) (p < 0.001), and an EQ-VAS score of 51.15 (SD: 19.70) (p = 0.006), compared to those with non-flares. Patients' flare status was a significant predictor of impaired HRQoL based on DLQI (p = 0.004), EQ-5D (p < 0.001) and EQ-VAS (p < 0.001) while higher BMI was associated with impaired EQ-VAS score (p = 0.001).</p><p><strong>Conclusion: </strong>The study demonstrates the substantial and dynamic burden of GPP, with a significant impairment in HRQoL among the patients in Malaysia, particularly during disease flares. These findings suggest the importance of prioritizing flares prevention and addressing the holistic needs of GPP patients to maximize their quality of life over the long term.</p>","PeriodicalId":19651,"journal":{"name":"Orphanet Journal of Rare Diseases","volume":"20 1","pages":"460"},"PeriodicalIF":3.5000,"publicationDate":"2025-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12392660/pdf/","citationCount":"0","resultStr":"{\"title\":\"Health-related quality of life of adults with generalized pustular psoriasis in Malaysia: a cross-sectional study.\",\"authors\":\"Xin Qian Seah, Siew Chin Ong, Mustapha Mohammed, Mei Ee Tay, Latha Selvarajah, Wooi Chiang Tan, Sook Yee Michelle Voo, Yen Loo Ting, Jyh Jong Tang, Azura Mohd Affandi\",\"doi\":\"10.1186/s13023-025-03820-2\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Generalized Pustular Psoriasis (GPP) is a rare but severe form of psoriasis, characterized by flares involving the sudden spread of erythema with sterile pustules, crusting, and scaling.</p><p><strong>Objective: </strong>The study aimed to assess both generic and dermatology-specific health-related quality of life (HRQoL) in patients with GPP across flare and non-flare stages in Malaysia.</p><p><strong>Methods: </strong>The study was a multicenter cross-sectional study conducted among patients diagnosed with GPP attending the General Hospitals under the Ministry of Health, Malaysia, between May 15, 2024, and November 30, 2024. The HRQoL of the GPP patients were assessed using the validated versions of the Dermatology Life Quality Index (DLQI) and EuroQol-5 Dimensions (EQ-5D). Data were statistically analysed using descriptive and inferential statistics using IBM SPSS version 28.0.</p><p><strong>Results: </strong>A total of fifty-four patients from six centres were enrolled into the study, with a mean (standard deviation, SD) age of 44.6 (SD: 15.5), the majority being female (n = 41, 75.9%). The mean (SD) scores of the DLQI and EQ-5D for the patients with GPP were 8.44 (SD: 7.12) and 0.74 (SD: 0.27), respectively. Thirteen patients (24%) were in their flares phase at the time of assessment. During the flares stage, patients experienced poorer quality of life, as indicated by a significantly higher mean DLQI score of 15.70 (SD: 7.33) (p < 0.001), lower EQ-5D score of 0.41 (SD: 0.30) (p < 0.001), and an EQ-VAS score of 51.15 (SD: 19.70) (p = 0.006), compared to those with non-flares. Patients' flare status was a significant predictor of impaired HRQoL based on DLQI (p = 0.004), EQ-5D (p < 0.001) and EQ-VAS (p < 0.001) while higher BMI was associated with impaired EQ-VAS score (p = 0.001).</p><p><strong>Conclusion: </strong>The study demonstrates the substantial and dynamic burden of GPP, with a significant impairment in HRQoL among the patients in Malaysia, particularly during disease flares. These findings suggest the importance of prioritizing flares prevention and addressing the holistic needs of GPP patients to maximize their quality of life over the long term.</p>\",\"PeriodicalId\":19651,\"journal\":{\"name\":\"Orphanet Journal of Rare Diseases\",\"volume\":\"20 1\",\"pages\":\"460\"},\"PeriodicalIF\":3.5000,\"publicationDate\":\"2025-08-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12392660/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Orphanet Journal of Rare Diseases\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1186/s13023-025-03820-2\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"GENETICS & HEREDITY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Orphanet Journal of Rare Diseases","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1186/s13023-025-03820-2","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"GENETICS & HEREDITY","Score":null,"Total":0}
Health-related quality of life of adults with generalized pustular psoriasis in Malaysia: a cross-sectional study.
Background: Generalized Pustular Psoriasis (GPP) is a rare but severe form of psoriasis, characterized by flares involving the sudden spread of erythema with sterile pustules, crusting, and scaling.
Objective: The study aimed to assess both generic and dermatology-specific health-related quality of life (HRQoL) in patients with GPP across flare and non-flare stages in Malaysia.
Methods: The study was a multicenter cross-sectional study conducted among patients diagnosed with GPP attending the General Hospitals under the Ministry of Health, Malaysia, between May 15, 2024, and November 30, 2024. The HRQoL of the GPP patients were assessed using the validated versions of the Dermatology Life Quality Index (DLQI) and EuroQol-5 Dimensions (EQ-5D). Data were statistically analysed using descriptive and inferential statistics using IBM SPSS version 28.0.
Results: A total of fifty-four patients from six centres were enrolled into the study, with a mean (standard deviation, SD) age of 44.6 (SD: 15.5), the majority being female (n = 41, 75.9%). The mean (SD) scores of the DLQI and EQ-5D for the patients with GPP were 8.44 (SD: 7.12) and 0.74 (SD: 0.27), respectively. Thirteen patients (24%) were in their flares phase at the time of assessment. During the flares stage, patients experienced poorer quality of life, as indicated by a significantly higher mean DLQI score of 15.70 (SD: 7.33) (p < 0.001), lower EQ-5D score of 0.41 (SD: 0.30) (p < 0.001), and an EQ-VAS score of 51.15 (SD: 19.70) (p = 0.006), compared to those with non-flares. Patients' flare status was a significant predictor of impaired HRQoL based on DLQI (p = 0.004), EQ-5D (p < 0.001) and EQ-VAS (p < 0.001) while higher BMI was associated with impaired EQ-VAS score (p = 0.001).
Conclusion: The study demonstrates the substantial and dynamic burden of GPP, with a significant impairment in HRQoL among the patients in Malaysia, particularly during disease flares. These findings suggest the importance of prioritizing flares prevention and addressing the holistic needs of GPP patients to maximize their quality of life over the long term.
期刊介绍:
Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.