Taiwanese Dermatological Association consensus recommendations for the diagnosis, treatment, and management of generalized pustular psoriasis
Journal
Dermatologica Sinica
Series/Report No.
Dermatologica Sinica
Journal Volume
42
Journal Issue
2
Start Page
98-109
ISSN
1027-8117
2223-330X
Date Issued
2024-01-01
Author(s)
Hsu, Chao-Kai
Huang, Yu-Huei
Chang, Chung-Hsing
Chen, Yi-Ju
Chiu, Tsu-Man
Chung, Wen-Hung
Jang, Chiau-Sheng
Lin, Shang-Hung
Lu, Chun-Wei
Wu, Nan-Lin
Yu, Sebastian
et al.,
DOI
10.4103/ds.DS-D-24-00070
Abstract
Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening skin disease characterized by relapsing and remitting flares of sterile neutrophilic pustules and systemic inflammation. The definition of GPP is inconsistent globally, with large discrepancies in clinical management. To provide clinical guidance on managing GPP, we conducted a systematic literature search for articles published within the last decade on PubMed and the Cochrane Library in October 2022 and held four consensus meetings with 12 Taiwanese dermatologists between October 2022 and July 2023. Upon review of 153 articles, we agreed to adopt the European Rare and Severe Psoriasis Expert Network GPP definition with additional clarifications on pustular flares in psoriatic plaques, circinate or annular lesions, and localized pustules. We also drafted a diagnostic algorithm to facilitate GPP diagnosis. Twenty-seven statements on GPP treatment reached consensus. We recommend using an oral retinoid or spesolimab injection for the first-line treatment in both acute (treating flares) and maintenance (preventing flares) settings in adults with GPP. For infants and juveniles with GPP, retinoids are recommended as a first-line treatment. Evidence for other conventional and investigational therapies was reviewed, and a treatment algorithm was proposed. We hope this consensus provides practical guidance for clinicians in Taiwan and helps improve outcomes for GPP patients.
SDGs
Publisher
Medknow
Type
journal article