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. 2025 Mar 18;192(4):706-716.
doi: 10.1093/bjd/ljae491.

Global Delphi consensus on treatment goals for generalized pustular psoriasis

Jonathan N Barker  1 , Emmylou Casanova  2 , Siew Eng Choon  3 , Peter Foley  4 , Hideki Fujita  5 , César Gonzalez  6 , Melinda Gooderham  7 , Slaheddine Marrakchi  8 , Luís Puig  9 , Ricardo Romiti  10 , Diamant Thaçi  11 , Min Zheng  12 , Bruce Strober  13 ; Partnering for Innovation and Excellence in Rare Skin Diseases (PIONEERS®) Working Group and the DELPHI Panel
Collaborators, Affiliations

Global Delphi consensus on treatment goals for generalized pustular psoriasis

Jonathan N Barker et al. Br J Dermatol. .

Abstract

Background: Generalized pustular psoriasis (GPP) is a chronic, systemic, neutrophilic inflammatory disease. A previous Delphi panel established areas of consensus on GPP, although patient perspectives were not included and aspects of treatment goals remained unclear.

Objectives: To identify and achieve consensus on refined, specific treatment goals for GPP treatment via a Delphi panel with patient participation.

Methods: Statements were generated based on a systematic literature review and revised by a Steering Committee. Statements were categorized into overarching principles, and short- and long-term treatment goals. A global panel of 30 dermatologists and 3 patient representatives voted in agreement or disagreement with each statement. Consensus was defined as ≥ 80% approval by the panellists.

Results: Consensus was reached in the first round of voting and ≥ 90% agreement was reached for 23 of 26 statements. In summary, GPP requires a timely, tailored treatment plan, co-developed by patients and physicians, that involves a multidisciplinary approach and addresses the complexity, heterogeneity and chronicity of the disease. Short-term treatment goals should include pustule clearance within 7 days and prevention of pustule recurrence, reduction of cutaneous symptom burden (-4 or more points on the Itch and Skin Pain Numeric Rating Scale), improvement in systemic symptoms (e.g. resolution of fever within 3 days of treatment initiation and reduced fatigue), prevention of life-threatening complications and progressive improvement of inflammatory biomarkers. In patients with comorbid psoriatic diseases, treatment decisions should prioritize GPP. Long-term treatment goals should include minimizing disease activity through flare prevention and symptom control between flares, sustained disease control, management of comorbidities and improvement in quality of life (QoL). Small differences in perception between patients and physicians regarding the importance of certain treatment goals (e.g. avoiding hair and/or nail loss to improve QoL), reflect the complexity of assessing treatment goals and emphasize the need for a patient-centred approach.

Conclusions: In the first global Delphi panel in GPP to include patient perspectives, consensus between dermatologists and patients was achieved on overarching principles of treatment, and short- and long-term treatment goals for GPP. These findings provide valuable insights for developing guidelines that consider the perspectives of patients and physicians in the treatment of GPP.

Plain language summary

Generalized pustular psoriasis (‘GPP’ for short) is a rare and life-long inflammatory disease that causes skin redness and blisters. People with GPP often experience a high temperature, tiredness and skin pain. The symptoms can suddenly become worse in episodes called ‘flares’. Until now, doctors have not had standard treatment goals for GPP, or agreed ways to measure if a medicine is working well. To determine what treatment goals are important to people with GPP and their doctors, a panel of 30 expert doctors and 3 patient representatives from 24 countries took part in a survey. The panel voted on 26 statements related to GPP treatment. The statements were prepared by a team of expert doctors. After 1 round of voting, the panel agreed on all the statements. Regarding GPP treatment, at least 32 out of 33 panellists agreed on each statement. The panel agreed that GPP is a complex, life-long disease, and that treatment should be started quickly, be tailored to each patient and involve doctors from other specialties, as well as dermatologists (skin doctors). Doctors and patient representatives gave different levels of importance to some treatment goals. For example, the 3 patient representatives all agreed that avoiding losing their hair/nails was important, but only 24 out of 30 doctors held the same view. This particular type of survey on GPP treatment goals was the first to include patient representatives. Panellists agreed on all short-term, long-term and overall treatment goals for GPP.

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Conflict of interest statement

Conflicts of interest: J.N.B. has received honoraria and/or research grants from AbbVie, Almirall, Amgen, Boehringer Ingelheim, Bristol Myers Squibb, Celgene, Eli Lilly, Janssen, LEO Pharma, Novartis, Samsung and Sun Pharmaceutical Industries. E.C. has received advisory board/speaker fees from Boehringer Ingelheim’s generalized pustular psoriasis activities through the International Federation of Psoriasis Association’s ambassador programme. S.E.C. declares paid activities as an advisor, speaker or consultant for AbbVie, Boehringer Ingelheim, Eli Lilly, Janssen, LEO Pharma, MSD, Novartis, Pfizer, Sanofi and UCB. P.F. has received grant support and/or has served on advisory boards and/or has served as a consultant and/or has received travel grants and/or has served as a speaker for or received honoraria from AbbVie, Akaal, Amgen, Arcutis, Argenx, Aslan, AstraZeneca, Boehringer Ingelheim, Botanix, Bristol Myers Squibb, Celgene, Celtaxsys, CSL Behring, Cutanea, Dermira, Evelo, Galderma, GenesisCare, Genentech, GlaxoSmithKline, Hexima, Janssen, Kymab, LEO Pharma, Lilly, Mayne Pharma, MedImmune, Melaseq/Geneseq, Merck, Novartis, Pfizer, Regeneron, Reistone, Roche, Sanofi, Sun Pharmaceutical Industries, Teva, UCB Pharma and Valeant. 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, Chugai Pharmaceutical Co., Eisai, Eli Lilly, Janssen, Japan Blood Products Organization, JMEC, Kaken, Kyorin, Kyowa Kirin, LEO Pharma, Maruho, Mitsubishi Tanabe, Nihon Pharmaceutical, Novartis, Sanofi, Sun Pharmaceutical Industries, Taiho, Torii, UCB and Ushio. C.G. has received consultancy/speaker’s honoraria from and/or participated in clinical trials sponsored by AbbVie, Amgen, Boehringer Ingelheim, Janssen, Lilly, Novartis and Pfizer. M.G. has been an investigator, speaker and/or advisor for AbbVie, Amgen, Akros, Arcutis, Bausch Health, Bristol Myers Squibb, Boehringer Ingelheim, Celgene, Dermira, Dermavant, Eli Lilly, Galderma, GlaxoSmithKline, Incyte, Janssen, Kyowa Kirin, LEO Pharma, MedImmune, Merck, Novartis, Pfizer, Regeneron, Roche, Sanofi-Genzyme, Sun Pharmaceutical Industries and UCB. S.M. declares paid consulting activities for Boehringer Ingelheim. L.P. has received consultancy/speaker’s honoraria from and/or participated in clinical trials sponsored by AbbVie, Almirall, Amgen, Biogen, Boehringer Ingelheim, Bristol Myers Squibb, Eli Lilly, Janssen, LEO Pharma, Novartis, Pfizer, Sandoz, Sanofi and UCB. R.R. declares receiving honoraria as a scientific consultant, speaker or clinical study investigator for AbbVie, Boehringer Ingelheim, Bristol Myers Squibb, Galderma, Janssen, Eli Lilly, LEO Pharma, Novartis, Pfizer, Pierre-Fabre, Sanofi, Teva and UCB. D.T. has served as a consultant, advisory board member and/or investigator for AbbVie, Almirall, Amgen, Beiersdorf, Boehringer Ingelheim, Bristol Myers Squibb, Celltrion, Eli Lilly, Janssen Cilag, LEO Pharma, L’Oréal, New Bridge, Novartis, Pfizer, Regeneron, Sanofi, Sun Pharmaceutical Industries and UCB. M.Z. has received grants, consulting fees and/or speaker’s fees from AbbVie, Boehringer Ingelheim, Janssen Cilag, LEO Pharma China, Novartis, Pfizer and Xian-Janssen. B.S. is a consultant and has received honoraria from AbbVie, Acelyrin, Alamar, Alumis, Almirall, Amgen, Arcutis, Arena, Aristea, Asana, Boehringer Ingelheim, Kangpu Biopharmaceuticals, Bristol Myers Squibb, Capital One, Celltrion, CorEvitas, Dermavant, Inmagene, Janssen, LEO Pharma, Eli Lilly, Maruho, Okura, Meiji Seika Pharma, Protagonist, Monte Carlo, Takeda, Novartis, Pfizer, UCB Pharma, Rapt, Regeneron, Sanofi-Genzyme, SG Cowen and Union Therapeutics. He has stock options in Connect Biopharma and Mindera Health, and has acted as a speaker for AbbVie, Arcutis, Dermavant, Eli Lilly, Incyte, Janssen, Regeneron and Sanofi-Genzyme. He is an investigator for and receives consulting fees as scientific co-director of the CorEvitas Psoriasis Registry and receives honoraria as editor-in-chief of the Journal of Psoriasis and Psoriatic Arthritis.

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