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Practice Guideline
. 2024 Dec;51(12):1579-1595.
doi: 10.1111/1346-8138.17471. Epub 2024 Oct 10.

Asia-Pacific consensus recommendations on the management of generalized pustular psoriasis

Siew Eng Choon  1 , Peter Anthony Foley  2   3 , Pravit Asawanonda  4 , Hideki Fujita  5 , Seong-Jin Jo  6 , Yu-Ling Shi  7   8 , Colin Theng  9 , Azura Mohd Affandi  10 , Chul Hwan Bang  11 , Maria Lorna Frez  12 , Huang Yu Huei  13   14 , Doanh Le Huu  15 , Tae-Gyun Kim  16 , Akimichi Morita  17 , Hazel H Oon  18   19 , Pablo Fernández-Peñas  20   21 , Natta Rajatanavin  22 , Suganthy Robinson  10 , Latha Selvarajah  23 , Tsen-Fang Tsai  24
Affiliations
Practice Guideline

Asia-Pacific consensus recommendations on the management of generalized pustular psoriasis

Siew Eng Choon et al. J Dermatol. 2024 Dec.

Abstract

Generalized pustular psoriasis (GPP) is a rare, chronic, heterogeneous, and potentially life-threatening disease characterized by primary, sterile, and macroscopically visible pustules with or without systemic symptoms. There are ethnic differences in the genetic mutations associated with GPP that might affect the clinical manifestations and treatment responses. Currently, there is limited evidence from the patient population in the Asia-Pacific (APAC) region, resulting in a general paucity of information on the effective management of patients with GPP in this region. This modified Delphi panel study aimed to identify current evidence and gain advanced insights to facilitate the development of a regionally tailored APAC consensus on the management of GPP. A systematic literature review (SLR) was conducted to identify published literature and develop consensus statements on (i) definition and clinical course, (ii) diagnosis of GPP, (iii) treatment outcomes, goals, and monitoring measures, and (iv) optimal management strategies and clinical practices. Statements were rated by a panel of dermatologists in two rounds, with the threshold for consensus at ≥80% agreement. Twenty experts from the APAC region reached consensus on 106 statements that were developed based on the SLR and experts' collective expertise. The experts agreed that GPP is a rare, severe, and potentially life-threatening condition that is distinct from plaque psoriasis. This consensus emphasized the importance of a tailored treatment strategy taking into account the GPP flare severity and each patient's unique clinical circumstances. The experts reached consensus on the severity classification of GPP flares and recommended first-line and maintenance treatment options for adult GPP, childhood GPP, and GPP in pregnancy. These consensus outcomes have been synthesized into treatment algorithms to guide dermatologists in the APAC region in their clinical decision-making processes.

Keywords: Asia‐Pacific; Delphi panel; consensus; generalized pustular psoriasis; management.

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Conflict of interest statement

S.E.C. declared paid activities as an advisor, speaker or consultant for AbbVie, Almirall, Boehringer Ingelheim, Eli Lilly, Janssen, Novartis, and Pfizer. P.A.F. declared receiving grant support from AbbVie, Amgen, Bristol Myers Squibb, Celgene, Eli Lilly, Galderma, Janssen, Leo Pharma, Merck, Novartis, Pfizer, Sanofi, Sun Pharma, and UCB; travel grants from AbbVie, Eli Lilly, Galderma, Janssen, Leo Pharma, Merck, Novartis, Pfizer, Roche, Sanofi, and Sun Pharma; and declared activities as an investigator for AbbVie, Amgen, Arcutis, Argenx, ASLAN, AstraZeneca, Boehringer Ingelheim, Botanix, Bristol Myers Squibb, Celgene, Celtaxsys, CSL, Cutanea, Dermira, Eli Lilly, Evelo Biosciences, Galderma, Genentech, Geneseq, GlaxoSmithKline, Hexima, Incyte, Janssen, Kymab, Leo Pharma, Merck, Novartis, Pfizer, Regeneron, Reistone, Roche, Sanofi, Sun Pharma, Takeda, Teva, UCB, Valeant, and ZaiLab; an advisory board member for AbbVie, Amgen, Aslan, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Eli Lilly, Galderma, GlaxoSmithKline, Janssen, Leo Pharma, Mayne Pharma, Novartis, Pfizer, Sanofi, Sun Pharma, UCB, and Valeant; a consultant for Aslan, Bristol Myers Squibb, Eli Lilly, Galderma, GenesisCare, Hexima, Janssen, Leo Pharma, Mayne Pharma, MedImmune, Novartis, Pfizer, Roche, and UCB; as a speaker or honoraria recipient from AbbVie, Amgen, Bristol Myers Squibb, Celgene, Eli Lilly, Galderma, GlaxoSmithKline, Janssen, Leo Pharma, Merck, Novartis, Pfizer, Roche, Sanofi, Sun Pharma, and Valeant. P.A. declared receiving honoraria or payment from Boehringer Ingelheim, Pfizer, Eli Lilly, Novartis and Janssen, and support for attending meetings and/or travel from Pfizer, Eli Lilly, and Novartis. H.F. has received honoraria or fees for serving on advisory boards, as a speaker and as a consultant, as well as grants as an investigator from AbbVie, Amgen, Boehringer Ingelheim, Celgene, Eisai, Eli Lilly, Janssen, Kyowa Kirin, LEO Pharma, Maruho, Mitsubishi‐Tanabe, Novartis, Sanofi, Sun Pharma, Taiho, Torii, UCB, and Ushio. H.F. is an Editorial Board member of Journal of Dermatology and to minimize bias, he was excluded from all editorial decision‐making related to the acceptance of this article for publication. S.J.J. declared activities as an advisor, speaker, or consultant for and/or received research grants from AbbVie, Boehringer Ingelheim, Bristol Myers Squibb, Celltrion Healthcare, Daewoong, Eli Lilly and Company, Green Cross Laboratories, Janssen Pharmaceuticals, Kolon Pharma, LEO Pharma, Novartis, Pfizer, Sanofi, UCB, and Yuhan. C.T. has served as a speaker or consultant for Janssen, AbbVie, Novartis, Amgen, and Boehringer Ingelheim. A.M.A. has received honoraria for serving as advisor and speaker for AbbVie, Boehringer Ingelheim, Janssen, Leo Pharma, Novartis, Pfizer, Sanofi and ZP Therapeutics. A.M.A. is also the principal investigator for clinical trials funded by Boehringer Ingelheim. C.H.B. declared paid activities as an advisor, speaker or consultant and/or received research grants from AbbVie, Boehringer Ingelheim, Bristol Myers Squibb, Celltrion, Eli Lilly, Janssen, Kolon Pharma, Leo Pharma, Novartis, Pfizer, and UCB. M.L.F. has received educational grants and honoraria or fees for serving as a member of advisory board, speaker for Johnson & Johnson, Novartis, Zuellig Pharma, and Leo Pharma. H.Y.H. has conducted clinical trials while serving as a principal investigator for AbbVie, Bristol‐Myers Squibb, Galderma, Eli Lilly, Novartis, Janssen‐Cilag Pharmaceutical, and Pfizer; received honoraria for serving as an advisory board member for Pfizer, AbbVie, and Celgene; and received speaking fees from AbbVie, Eli Lilly, and Novartis. A.M. has received research grants, consultancy fees, and/or speaker's fees from AbbVie, Amgen, Boehringer Ingelheim, Bristol‐Myers Squibb, Eisai, Eli Lilly Japan, Janssen Pharmaceutical, Kyowa Kirin, LEO Pharma, Maruho, Minophagen Pharmaceutical, Mitsubishi Tanabe Pharma, Nippon Kayaku, Novartis, Pfizer Japan, Sun Pharma Japan, Taiho Pharmaceutical, Torii Pharmaceutical, UCB Japan, and Ushio. H.H.O. has served as a speaker, advisory board member, and researcher for AbbVie, Galderma, Janssen and Novartis. She has also been a clinical investigator for Pfizer and Sanofi, advisory board member for Amgen as well as a speaker and advisory board member for Boehringer Ingelheim and Eli Lilly. P.F.P. has served on advisory boards for AbbVie, Amgen, Boehringer Ingelheim, Bristol Myers Squib, Celgene, Janssen, LEO Pharma, Eli Lilly and Company, Merck, Merck Sharp & Dohme, Novartis, Roche, Sanofi, and Sun Pharma; has given educational lectures for AbbVie, Amgen, Avene, Eli Lilly, Galderma, Janssen, La Roche‐Posay, LEO Pharma, Merck, Novartis, Pfizer, Roche, Sanofi, Schering Plough, Sun Pharma, and UCB Pharma; has conducted clinical trials for AbbVie, Amgen, Arena, AstraZeneca, Boehringer Ingelheim, Bristol Myers Squibb, CSL, Dermira, Eisai, Eli Lilly and Company, Galderma, GlaxoSmithKline, Janssen, Jiangsu Hengrui, Kyowa Hakko Kirin, LEO Pharma, miRagen, Novartis, OncoSec, Pfizer, Regeneron, Roche, Sun Pharma, UCB Pharma, and Xoma. L.S. declared paid activities as an advisor/speaker for AbbVie, Boehringer Ingelheim, DKSH, Eli Lilly, Janssen, Leo Pharma, MSD, Novartis, Sanofi, and Zuellig Pharma. T.F.T. has conducted clinical trials or received honoraria for serving as a consultant for AbbVie, AnaptysBio, Boehringer Ingelheim, Bristol‐Myers Squibb, Celgene, Eli Lilly, Galderma, GSK, Janssen‐Cilag, Leo Pharma, Merck Sharp & Dohme, Novartis International, Pfizer, PharmaEssentia, Sanofi, Sun Pharma, and UCB Pharma. Y.S., D.L.H., T.G.K., N.R., and S.R. did not have conflicts of interests to disclose.

Hideki Fujita is an Editorial Board member of Journal of Dermatology and a co‐author of this article. To minimize bias, he was excluded from all editorial decision‐making related to the acceptance of this article for publication.

Figures

FIGURE 1
FIGURE 1
Overview of the consensus development process. *Via Survey Monkey platform. Abbreviation: CSDG, Consensus Statement Development Group.
FIGURE 2
FIGURE 2
Diagnostic algorithm for patients with GPP. Abbreviations: AGEP, acute generalized exanthematous pustulosis; AP1S3, adaptor related protein complex 1 subunit sigma 3; CARD14, caspase recruitment domain family member 14; GPP, generalized pustular psoriasis; IF, immunofluorescence; IgA, immunoglobulin A; MPO, myeloperoxidase; SERPINA3, serpin family A member 3.
FIGURE 3
FIGURE 3
Treatment algorithm for the management of adults with GPP flares. *The severity classification of GPP flares is intended as a guide only and should not be used to limit treatment access. Treatment choice should follow local guidelines. †Etretinate if acitretin is not available. ‡3.5–5 mg/kg. §0.5–1 mg/kg. 0.125–0.5 mg/kg. Abbreviations: BSA, body surface area; GPP, generalized pustular psoriasis; GPPGA, Generalized Pustular Psoriasis Physician Global Assessment; IL, interleukin; TNF, tumor necrosis factor.
FIGURE 4
FIGURE 4
Treatment algorithm for the management of childhood GPP. *Etretinate if acitretin is not available. Abbreviations: GPP, generalized pustular psoriasis; IL, interleukin; TNF, tumor necrosis factor.
FIGURE 5
FIGURE 5
Treatment algorithm for the management of GPP in pregnancy. Abbreviations: BCG, Bacillus Calmette–Guérin; GPP, generalized pustular psoriasis.
See this image and copyright information in PMC

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