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. 2023;239(3):345-354.
doi: 10.1159/000529274. Epub 2023 Feb 16.

Clinical Characteristics and Outcomes of Generalized Pustular Psoriasis Flares

Siew Eng Choon  1 , Mark G Lebwohl  2 , Hamida Turki  3 , Min Zheng  4 , A David Burden  5 , Ling Li  6 , Manuel Quaresma  7 , Christian Thoma  8 , Hervé Bachelez  9
Affiliations

Clinical Characteristics and Outcomes of Generalized Pustular Psoriasis Flares

Siew Eng Choon et al. Dermatology. 2023.

Abstract

Background: Generalized pustular psoriasis (GPP) is a rare, neutrophilic skin disease that can become life-threatening if flares are untreated. There are limited data describing the characteristics and clinical course of GPP disease flares with current treatment options.

Objective: The aim of the study was to describe the characteristics and outcomes of GPP flares using historical medical information from patients enrolled in the Effisayil™ 1 trial.

Methods: Investigators collected retrospective medical data characterizing patients' GPP flares prior to clinical trial enrollment. Data on overall historical flares were collected, as well as information on patients' typical, most severe, and longest past flares. This included data on systemic symptoms, flare duration, treatment, hospitalization, and time to clearance of skin lesions.

Results: In this cohort (N = 53), patients with GPP experienced a mean of 3.4 flares per year. Flares were painful, associated with systemic symptoms, and often triggered by stress, infections, or treatment withdrawal. Resolution of flares was longer than 3 weeks in 57.1%, 71.0%, and 85.7% of documented (or identified) typical, most severe, and longest flares, respectively. GPP flares led to patient hospitalization in 35.1%, 74.2%, and 64.3% of patients for their typical, most severe, and longest flares, respectively. For the majority of patients, pustules took up to 2 weeks to clear for a typical flare and 3-8 weeks to clear for the most severe and longest flares.

Conclusion: Our findings highlight that current treatment options are slow to control GPP flares and provide context for assessing the efficacy of new therapeutic strategies in patients with a GPP flare.

Keywords: Clinical research; Clinical trial; Generalized pustular psoriasis; Psoriasis.

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Conflict of interest statement

The authors did not receive payment related to the development of this manuscript. Boehringer Ingelheim was given the opportunity to review the manuscript for medical and scientific accuracy as well as intellectual property considerations. Siew Eng Choon declares paid activities as an advisor, speaker, or consultant for AbbVie, Boehringer Ingelheim, Eli Lilly, Janssen, LEO Pharma, MSD, Novartis, Pfizer, Sanofi, and UCB. Mark G. Lebwohl is an employee of Mount Sinai, has received research funds from AbbVie, Amgen, Arcutis, Avotres, Boehringer Ingelheim, Cara Therapeutics, Dermavant Sciences, Eli Lilly, Incyte, Janssen Research & Development, LLC, Ortho Dermatologics, Regeneron, and UCB, and is a consultant for Aditum Bio, Almirall, AltruBio, AnaptysBio, Arcutis, Arena Pharmaceuticals, Aristea Therapeutics, Arrive Technologies, Avotres Therapeutics, BiomX, Boehringer Ingelheim, Brickell Biotech, Bristol Myers Squibb, Cara Therapeutics, Castle Biosciences, CorEvitas, Dermavant Sciences, Dr. Reddy's Laboratories, Evelo Biosciences, Evommune, Facilitation of International Dermatology Education, Forte Biosciences, Foundation for Research and Education in Dermatology, Helsinn Therapeutics, Hexima, LEO Pharma, Meiji Seika Pharma, Mindera, Pfizer, Seanergy, and Verrica. Hamida Turki declares paid consulting activities for Boehringer Ingelheim. Min Zheng declares receiving grants, consulting fees, and/or speaker's fees from AbbVie, Boehringer Ingelheim, Janssen-Cilag, LEO Pharma China, Novartis, Pfizer, and Xian Janssen. A. David Burden declares paid consulting activities for AbbVie, Almirall, Boehringer Ingelheim, Celgene, Eli Lilly, Janssen, LEO Pharma, Novartis, and UCB. Ling Li, Manuel Quaresma, and Christian Thoma are employees of Boehringer Ingelheim. Hervé Bachelez declares paid consulting activities for AbbVie, Almirall, AnaptysBio, Aristea Therapeutics, BIOCAD, Boehringer Ingelheim, Bristol Myers Squibb, Dermavant Sciences, Eli Lilly, Janssen, Kyowa Kirin, LEO Pharma, Novartis, UCB, and Xion Pharmaceuticals; grant support from Boehringer Ingelheim, Bristol Myers Squibb, Janssen, LEO Pharma, Novartis, and Pfizer; and participation in a data safety monitoring board/advisory board for Avillion.

Figures

Fig. 1
Fig. 1
Duration of past flares. The doughnut plots show for a typical, most severe, and longest past flare the proportion of patients (%) with different flare durations. Flare duration is categorized as <1 week, 1–2 weeks, 3–4 weeks, 5–8 weeks, 9–12 weeks, and >12 weeks. Typical past flare, n = 35; most severe past flare, n = 31; longest past flare, n = 14.
Fig. 2
Fig. 2
Duration of hospitalization for past flares. The bar chart shows the proportion of patients (%) with different durations of hospitalization for a typical, most severe, and longest past flare. Duration of hospitalization is categorized as <1 week, 1–2 weeks, and 3–4 weeks. Hospitalization data were unknown in 4 patients (10.8%) for a typical past flare. Typical past flare, n = 37; most severe past flare, n = 31; longest past flare, n = 14.
Fig. 3
Fig. 3
Treatment of past flares. The doughnut plots show the proportion of patients (%) who have received prior treatment with systemic therapy, topical therapy, no therapy, or unknown, for their typical, most severe, and longest past flares. Typical past flare, n = 37; most severe past flare, n = 31; longest past flare, n = 14.
Fig. 4
Fig. 4
Time to clearance of pustules, erythema, and scaling. The bar charts show the proportion of patients (%) with different amounts of time taken to achieve clearance of pustules (a), erythema (b), and scaling (c) for their typical, most severe, and longest past flares. Time to clearance is categorized as <1 week, 1–2 weeks, 3–4 weeks, 5–8 weeks, 9–12 weeks, and >12 weeks. *Data available for 28 patients; †data available for 28 patients; ‡data available for 11 patients; §data available for 27 patients; data available for 29 patients.
See this image and copyright information in PMC

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