Generalized pustular psoriasis (GPP) is a rare, chronic inflammatory skin condition that is potentially life-threatening. Accumulating evidence suggests that GPP is distinct from the more common plaque psoriasis in terms of aetiology and clinical and dermatological features. The aim of this study was to describe the epidemiology, comorbidities, mortality and healthcare resource use (HCRU) in patients with GPP compared with those with plaque psoriasis in England. A retrospective cohort study of the Clinical Practice Research Datalink (CPRD) Aurum database linked with hospital and mortality data, between 2008 and 2019, was conducted, and HCRU between 2015 and 2019 was calculated. Prevalence rates, comorbidities and survival rates were estimated for GPP and plaque psoriasis. Diagnoses were based on CPRD medical codes and International Classification of Diseases 10th Revision codes (L40.1 for GPP, and L40.0 for plaque psoriasis). In total, 373 patients with GPP (62.5% female) and 224 223 patients with plaque psoriasis (49.4% female) were identified. The mean age (SD) was 55.9 years (18.6) for GPP, and 48.5 years (19.1) for patients with plaque psoriasis. A higher proportion of patients with GPP had at least one comorbidity, compared with plaque psoriasis (76.4% vs. 55.9%, respectively). From 2008 to 2019, the prevalence rates per 100 000 persons ranged from 1.61 to 3.0 for GPP, and from 1771 to 1904 for plaque psoriasis. In total, 78 of the 373 (20.9%) patients with GPP died during the study period, compared with 20 771 of 224 223 (9.3%) patients with plaque psoriasis. Compared with plaque psoriasis, survival rates were lower for patients with GPP, particularly those who were over 55 years old and those with a history of at least one comorbidity. Overall, 297 patients with GPP and 181 925 with plaque psoriasis were included in HCRU analysis. Hospitalizations, outpatient visits and accident and emergency visits were more frequent in patients with GPP compared with plaque psoriasis (78% vs. 47%, 92% vs. 75% and 71% vs. 52%, respectively). Our results support previous findings that GPP is a distinct disease from plaque psoriasis with a different epidemiology. Compared with plaque psoriasis, patients with GPP had higher mortality and were found to have higher disease burden, demonstrating a high unmet need in this patient group. The study was supported and funded by Boehringer Ingelheim. Boehringer Ingelheim was given the opportunity to review the manuscript for medical and scientific accuracy, as well as intellectual property considerations.
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Frysz et al. (2024) studied this question.
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