Clinical course and disease burden of patients with generalized pustular psoriasis in Portugal: a multicenter retrospective cohort study

Abstract

Objectives

Generalized pustular psoriasis (GPP) is a rare, life-threatening skin inflammatory disorder. This study aimed to describe the disease course, treatment strategies, and healthcare utilization among patients with GPP in Portugal.

Methods

This multicentric, observational, retrospective study included consecutive adult patients with GPP undergoing a dermatology evaluation in different reporting institutions by experienced dermatologists between 2002 and 2023.

Results

A total of 59 patients were assessed. Most of the cohort had a previous history of plaque psoriasis (71%) and 83% presented at least one comorbidity. At the initial encounter, 64% of the cohort needed hospitalization. Systemic involvement was common, including fever (37%), and elevated white blood cell count and erythrocyte sedimentation rate/C-reactive protein (49%). Nearly, 73% of patients initiated systemic drugs, and 70% had to discontinue the first treatment. During the study, 98% of patients experienced at least one flare. At the last visit, 3.4% of patients had died, and 71.2% exhibited signs of active disease despite undergoing treatment.

Conclusions

Our study demonstrates that GPP is a chronic, debilitating condition associated with systemic involvement, frequent flares, and hospitalizations, despite receiving multiple systemic treatments. Improved disease awareness and new treatments are needed to improve patient care and decrease the burden of the disease.

Keywords:

• Biologics
• burden
• flare
• generalized pustular psoriasis
• IL-36

Author contributions statement

Concept and design: TT, JA, AB, JA, DB, JR, DS, CC, AGP, AAM, JFM, BVG, PP, GMP, PQ, FMB, LT, SM, MJPL, HO, PV, JTS, PF, RTB

Analysis and interpretation of data: TT, JA, LT

Drafting of the manuscript: TT, JA

Critical revision of the manuscript for important intellectual content: TT, JA, AB, JA, DB, JR, DS, CC, AGP, AAM, JFM, BVG, PP, GMP, PQ, FMB, LT, SM, MJPL, HO, PV, JTS, PF, RTB

Final approval: TT, JA, AB, JA, DB, JR, DS, CC, AGP, AAM, JFM, BVG, PP, GMP, PQ, FMB, LT, SM, MJPL, HO, PV, JTS, PF, RTB

All authors agree to be accountable for all aspects of the work.

Disclosure statement

Tiago Torres has received consultancy and/or speaker’s honoraria from and/or participated in clinical trials sponsored by AbbVie, Almirall, Amgen, Arena Pharmaceuticals, Biocad, Biogen, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Fresenius Kabi, Janssen, LEO Pharma, Eli Lilly, MSD, Mylan, Novartis,

Pfizer, Samsung-Bioepis, Sanofi-Genzyme, Sandoz and UCB.

Pedro Ponte has received consultancy and/or speaker’s honoraria from and/or participated in clinical trials sponsored by Leo, Janssen, Abbvie, Novartis, Pfizer, Lilly, and Almirall.

Gabriela Marques Pinto has received consultancy and/or speaker’s honoraria from and/or participated in clinical trials sponsored by Abbvie, Almirall, Janssen, LEO Pharma, Lilly, and Novartis.

Maria João Paiva Lopes has received consultancy and/or speaker’s honoraria from and/or participated in clinical trials sponsored by AbbVie, Almirall, Boehringer Ingelheim, Janssen, LEO Pharma, Eli Lilly, Mylan, Novartis, Pfizer, Sanofi-Genzyme, and Viatris.

The remaining authors have no conflicts of interest.

Additional information

Funding

Research supported by grants from Boehringer Ingelheim Portugal. Boehringer Ingelheim was given the opportunity to review the manuscript for medical and scientific accuracy as it relates to Boehringer Ingelheim substances, as well as intellectual property considerations. The funding organization had no role in the design or conduct of this research.