Global Delphi consensus on treatment goals for generalized pustular psoriasis
Authors: Barker Jonathan N , Casanova Emmylou , Choon Siew Eng , Foley Peter , Fujita Hideki , Gonzalez César , Gooderham Melinda , Marrakchi Slaheddine , Puig Luís , Romiti Ricardo , Thaçi Diamant , Zheng Min , Strober Bruce ,   Partnering for Innovation and Excellence in Rare Skin Diseases (PIONEERS®) Working Group and the DELPHI Panel, Magariños Gabriel , Reich Adam , Nakano Juliana , Gao Xinghua , Zhang Furen , El Sayed Mahira , Welzel Julia , Gisondi Paolo , Che Yaacob Yuzlina Binti , Prignano Francesca , Tada Yayoi , Jin Jo Seong , van der Kerkhof Peter , Casanova Emmylou , Rivera Raquel , Schmitt-Egenolf Marcus , Tsai Tsen-Fang , Bulbul Baskan Emel , Burden David , Mostaghimi Arash , Reisner Dale , Eyerich Kilian , Stratigos Alexandros , Pavlovsky Lev , Villanueva Quintero Delfina Guadalupe , Imafuku Shinichi , Becker Jennifer , Affandi Azura , Cortés Carolina , Manuel Carrascosa Jose , Wiseman Marni , Viguier Manuelle-Anne , Bhutani Tina 
Published: January 2025

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.