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. 2023 Mar 27;11(4):740.
doi: 10.3390/vaccines11040740.

Characteristics of Patients Experiencing a Flare of Generalized Pustular Psoriasis: A Multicenter Observational Study

Francesco Bellinato  1 , Paolo Gisondi  1 , Angelo Valerio Marzano  2   3 , Stefano Piaserico  4 , Clara De Simone  5   6 , Giovanni Damiani  7   8 , Giuseppe Argenziano  9 , Marina Venturini  10 , Paolo Dapavo  11 , Antonio Costanzo  12   13 , Matteo Megna  14 , Francesca Prignano  15 , Martina Burlando  16   17 , Francesca Satolli  18 , Andrea Carugno  19   20 , Elena Pezzolo  21 , Marco Romanelli  22 , Aldo Cuccia  23 , Giampiero Girolomoni  1
Affiliations

Characteristics of Patients Experiencing a Flare of Generalized Pustular Psoriasis: A Multicenter Observational Study

Francesco Bellinato et al. Vaccines (Basel). .

Abstract

Background: Generalized pustular psoriasis (GPP) is a rare, severe inflammatory skin disease characterized by recurrent episodes of flares. Characteristics of patients experiencing a flare are hardly described in a real-life setting. The aim of the study is to investigate the clinical characteristics of patients experiencing a flare of GPP.

Methods: Multicenter retrospective observational study on consecutive patients experiencing a flare of GPP between 2018 and 2022. Disease severity and quality of life were assessed by Generalized Pustular Psoriasis Area, Body Surface Area (BSA), and Severity Index (GPPASI), and Dermatology life quality index (DLQI) questionnaire, respectively. Visual analogue scale (VAS) of itch and pain, triggers, complications, comorbidities, pharmacological therapies, and outcome were collected.

Results: A total of 66 patients, 45 (68.2%) females, mean age 58.1 ± 14.9 years, were included. The GPPASI, BSA, and DLQI were 22.9 ± 13.5 (mean ± standard deviation), 47.9 ± 29.1, and 21.0 ± 5.0, respectively. The VAS of itch and pain were 6.2 ± 3.3 and 6.2 ± 3.0, respectively. Fever (>38 °C) and leukocytosis (WBC > 12 × 109/L) were found in 26 (39.4%) and 39 (59.1%) patients, respectively. Precipitating triggers were identified in 24 (36.3%) and included infections (15.9%), drugs (10.6%), stressful life events (7.6%), and corticosteroids withdrawal (3.0%). Fourteen (21.2%) patients were hospitalized because of complications including infections in 9 (13.6%) leading to death in one case and hepatitis in 3 (4.5%).

Conclusions: GPP flares can be severe and cause severe pain and itch with significant impact on the quality of life. In about one-third of patients the flare may have a persistent course and, with complications, lead to hospitalization.

Keywords: flare; generalized pustular psoriasis; psoriasis.

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Conflict of interest statement

The Authors declare no conflict of interest. G. Damiani received grants from Abbvie, Almirall, Pfizer, and Novartis, and was speaker for Abbvie, Almirall, Pfizer, Novartis, Bristol-Myers Squibb, and Leo Pharma; G. Girolomoni has received personal fees from AbbVie, Abiogen, Almirall, Amgen, Boehringer-Ingelheim, Bristol-Myers Squibb, Eli-Lilly, Leo Pharma, Merck Serono, Novartis, Pfizer, Samsung, and Sanofi. E. Pezzolo has received personal fees from Abbvie, Sanofi, Leo Pharma, Almirall, Novartis, and Janssen. A. V. Marzano reports consultancy/advisory boards disease-relevant honoraria from AbbVie, Boehringer-Ingelheim, Novartis, Pfizer, Sanofi, and UCB; M. Megna acted as a speaker or consultant for Abbvie, Almirall, Amgen, Eli Lilly, Janssen, Leo Pharma, Novartis, and UCB. C.De Simone has received personal fees from AbbVie, Alfasigma, Almirall, Amgen, Boehringer-Ingelheim, Eli Lilly, Janssen, Leo Pharma, Novartis, Sanofi, and UCB Pharma. M. Venturini has received personal fees from AbbVie, Almirall, Amgen, Boehringer-Ingelheim, Eli Lilly, Janssen, Leo Pharma, Novartis, and UCB Pharma. M. Burlando has received personal fees from AbbVie, Almirall, Amgen, Boehringer-Ingelheim, Eli Lilly, Janssen, Leo Pharma, Novartis, and UCB Pharma. The lead author (the manuscript’s guarantor) affirms that the manuscript is an honest, accurate, and transparent account of the study being reported; that no important aspects of the study have been omitted; and that any discrepancies from the study as planned (and, if relevant, registered) have been explained.

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