Health‐related quality of life in patients with generalized pustular psoriasis: A systematic literature review

Abstract

Generalised pustular psoriasis (GPP) is a rare, chronic, neutrophilic inflammatory skin disease characterised by episodes of widespread eruption of sterile, macroscopic pustules that can be accompanied by systemic inflammation and symptoms. A systematic literature review and narrative synthesis were conducted to determine the impact of GPP on patients’ health‐related quality of life (HRQoL) and patient‐reported severity of symptoms, and to compare its impact to patients with plaque psoriasis (plaque PsO). Searches were undertaken in Embase, MEDLINE, and the Cochrane Library from January 1st, 2002 to September 15th, 2022. Screening was carried out by two reviewers independently. Outcome measures included generic (e.g. EQ‐5D, SF‐36) and dermatology‐specific (e.g. DLQI) clinical outcome assessments, and other relevant patient‐reported outcome measures (PROMs) (e.g. severity of pain measured by a numerical rating scale). Overall, 20 studies were found to be eligible for inclusion, of which seven also had data for plaque PsO. The DLQI was the most frequently reported outcome measure (16/20 studies). When reported, mean DLQI(SD) scores varied from 5.7 (1.2) to 15.8 (9.6) across the studies, indicating a moderate to very large effect on HRQoL; the wide range of scores and large SDs were explained by the small population sizes (n ≤12 for all studies except two). Similar ranges and large SDs were also observed for other measures within individual studies. However, in general, people with GPP reported a greater impact of their skin condition on HRQoL, when compared to people with plaque PsO (i.e. higher DLQI scores) and higher severity for itch, pain and fatigue. This systematic review highlighted the need for studies with a larger population size, a better understanding of the impact of cutaneous and extracutaneous symptoms and co‐morbidities on HRQoL during and between GPP flares, and outcome measures specifically tailored to the unique symptoms and the natural course/history of GPP.

Clinical and Disease Burden of Patients with Generalized Pustular Psoriasis: A Review of Real-World Evidence

“…Further data describing the acute and long-term burden of GPP, and the health economic pathways and burden of disease, in various populations have been published recently [ 53 , 57 – 60 ]. However, there remains an unmet need for improved guidelines for GPP diagnosis and management, improved recognition and understanding of GPP by HCPs, and multi-level support involving primary care, emergency care, and dermatologists [ 61 ].…”

Development and validation of a prognostic model for predicting flares in generalized pustular psoriasis

“…2 The unpredictability of GPP flare presents an obstacle to effective long-term management and leads to considerable clinical, financial and psychological strain for patients. 3,4 Our previous studies and a recent clinical trial showed that flares are highly prevalent among GPP patients, underscoring the significant importance of predicting and preventing GPP flares. 5,6 Therefore, we conducted this study to elucidate the risk factors of GPP flare, and construct a predictive model grounded in the clinical characteristics of patients to forecast the occurrence of GPP flares.…”

Health-related quality of life in patients with generalized pustular psoriasis – a Swedish register study

“…A recent systemic literature review identified 20 studies on HRQoL in patients with GPP [ 18 ], of which seven related to patients with GPP and plaques psoriasis and 13 related to patients with GPP only. Most studies addressed dermatological-specific HRQoL, while few addressed general HRQoL.…”