Affiliation:
1. Hospital Sultanah Aminah Johor Bahru, Clinical School Johor Bahru Monash University Malaysia Johor Bahru Malaysia
2. Clínica Dermacross Santiago Chile
3. Department of Dermatology Medical University of Graz Graz Austria
4. Boehringer Ingelheim International GmBH Ingelheim am Rhein Germany
5. Symmetron London UK
6. Boehringer Ingelheim Pharmaceuticals, Inc. Ridgefield Connecticut USA
7. Icahn School of Medicine at Mount Sinai New York New York USA
Abstract
AbstractGeneralized pustular psoriasis (GPP) is a rare, chronic, neutrophilic inflammatory skin disease characterized 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 1 January 2002 to 15 September 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 out of 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 comorbidities on HRQoL during and between GPP flares, and outcome measures specifically tailored to the unique symptoms and the natural course/history of GPP.
Subject
Infectious Diseases,Dermatology
Cited by
1 articles.
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