Generalized Pustular Psoriasis May Have Higher Mortality than Plaque Psoriasis

According to a recent observational claims analysis study, all-cause mortality is higher among patients with generalized pustular psoriasis (GPP) than among patients with plaque psoriasis.

While distinct from plaque psoriasis, GPP often exhibits a strong correlation and frequently associates with prior plaque psoriasis conditions. The disease’s course is heterogeneous and unpredictable, as flares can vary between patients in frequency, duration, and severity. Additional symptoms may also persist despite therapeutics.2

GPP mortality is most associated with complications such as sepsis, heart failure, and acute respiratory distress. However, investigators have noted the need for further data to further a comprehensive understanding of the disease burden and mortality risk associated with GPP.1

“This information is crucial for advancing the development of innovative treatments capable of addressing both morbidity and mortality effectively,” wrote Alice Gottlieb, MD, PhD, professor of dermatology and director of clinical trials at UTSW Medical Center, and colleagues. “The objective of this study was to compare all-cause mortality among patients with GPP to matched populations of patients with plaque [psoriasis] and the general population without GPP or [psoriasis].”1

Investigators included patients with GPP or plaque psoriasis who had ≥1 medical claim with an International Classification of Disease, 10th revision diagnosis from 2016 to 2019, or ≥2 outpatient claims ≥30 days apart from 2015 to 2020. Patients were then divided into 5 cohorts: those with only GPP, those with GPP and plaque psoriasis, those with only plaque psoriasis, a combined cohort of those with GPP and those with GPP and plaque psoriasis, and a general population cohort.1

All-cause mortality was then assessed across 2 timeframes: a 365-day post-index diagnosis period and a maximum follow-up duration for each patient, which extended until study conclusion or the patient experienced an event.1

In total, 1246 patients were included in the GPP only cohort, 1384 in the GPP and plaque psoriasis cohort, 2630 in the combined GPP cohort, 127,540 in the plaque psoriasis only cohort, and 19,641,441 in the general population cohort. Maximum follow-up ranged from 36.14 to 41.28 months (3.01-3.44 years).1

At the 365-day follow-up, the combined GPP cohort demonstrated a substantially higher mortality risk compared to the plaque psoriasis only and general population cohorts (HR, 2.57; 95% CI, 1.47-4.49; P <.001; HR, 6.3; 95% CI, 2.97-13.34; P <.001, respectively). Additionally, the GPP and plaque psoriasis cohort displayed an elevated risk compared to both the plaque psoriasis only and general population cohorts (HR, 3.37; 95% CI, 1.47-7.69; P = .004; HR, 5.04; 95% CI, 1.96-13; P = .001, respectively). The plaque psoriasis only cohort also had a higher mortality risk than the general population cohort (HR, 3.32; 95% CI, 2.84-3.89; P <.001).1

At the maximum follow-up period, the GPP only and GPP combination cohorts showed mortality risk over 1.5 times higher than the plaque psoriasis only cohort (HR, 1.55; 95% CI, 1.14-2.11; P = .005; HR, 1.61; 95% CI, 1.3-1.99; P <.001, respectively). The GPP combination cohort also demonstrated a mortality risk nearly 5 times the general population cohort (HR, 4.8; 95% CI, 3.59-6.42; P <.001). The GPP and plaque psoriasis cohort also surpassed 1.5 times that of the plaque psoriasis only cohort (HR, 1.59; 95% CI, 1.19-2.14; P = .002).1

Investigators noted, while this study indicates a significantly higher mortality risk for patients with GPP than for those with plaque psoriasis, the comparison was only statistically significant at the maximum follow-up period. The GPP cohort exhibited a 1% mortality rate at 365 days and a 5.7% rate at maximum follow-up. This is consistent with more recent studies, which suggest a GPP mortality rate between 2% and 16%.1

“These findings fill a significant gap in the existing literature and highlight both the need for increased awareness of the mortality burden in GPP and the need for continuous treatment options for patients with GPP both during and after flares,” Gottlieb and colleagues wrote.1

References

1. Gottlieb AB, Crooke Kwiatkowski H, Semeco J, Lakshminarasimhan B, Strober B, Lebwohl M. All-Cause Mortality is Higher in Generalized Pustular Psoriasis (GPP) than Plaque Psoriasis and the General Population: A US-Based Claims Analysis. J Psoriasis Psoriatic Arthritis. Published online May 30, 2025. doi:10.1177/24755303251344155

2. Crowley JJ, Pariser DM, Yamauchi PS. A brief guide to pustular psoriasis for primary care providers. Postgrad Med. 2021;133(3):330-344. doi:10.1080/00325481.2020.1831315