International Consensus Definition and Diagnostic Criteria for Generalized Pustular Psoriasis from the International Psoriasis Council

Siew Eng Choon*, Peter Van De Kerkhof, Johann E. Gudjonsson, Claudia De La Cruz, Jonathan Barker, Akimichi Morita, Ricardo Romiti, Azura Mohd Affandi, Pravit Asawanonda, Arthur David Burden, César Gonzalez, Slaheddine Marrakchi, Mohammad Rafiqul Mowla, Yukari Okubo, Hazel H. Oon, Tadashi Terui, Tsen Fang Tsai, Kristina Callis-Duffin, Hideki Fujita, Seong Jin JoJoseph Merola, Ulrich Mrowietz, Lluïs Puig, Diamant Thaçi, Margarita Velásquez, Matthias Augustine, Mahira El Sayed, Alexander A. Navarini, Andrew Pink, Jörg Prinz, Hamida Turki, Renata Magalhães, Francesca Capon, Hervé Bachelez

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

5 Citations (Scopus)

Abstract

Importance: Generalized pustular psoriasis (GPP) lacks internationally accepted definitions and diagnostic criteria, impeding timely diagnosis and treatment and hindering cross-regional clinical and epidemiological study comparisons. Objective: To develop an international consensus definition and diagnostic criteria for GPP using the modified Delphi method. Evidence Review: The rarity of GPP presents a challenge in acquiring comprehensive published clinical data necessary for developing standardized definition and criteria. Instead of relying on a literature search, 43 statements that comprehensively addressed the fundamental aspects of the definitions and diagnostic criteria for GPP were formulated based on expert reviews of 64 challenging GPP cases. These statements were presented to a panel of 33 global GPP experts for voting, discussion, and refinements in 2 virtual consensus meetings. Consensus during voting was defined as at least 80% agreement; the definition and diagnostic criteria were accepted by all panelists after voting and in-depth discussion. Findings: In the first and second modified Delphi round, 30 (91%) and 25 (76%) experts participated. In the initial Delphi round, consensus was achieved for 53% of the statements, leading to the approval of 23 statements that were utilized to develop the proposed definitions and diagnostic criteria for GPP. During the second Delphi round, the final definition established was, "Generalized Pustular Psoriasis is a systemic inflammatory disease characterized by cutaneous erythema and macroscopically visible sterile pustules." It can occur with or without systemic symptoms, other psoriasis types, and laboratory abnormalities. GPP may manifest as an acute form with widespread pustules or a subacute variant with an annular phenotype. The identified essential criterion was, "Macroscopically visible sterile pustules on erythematous base and not restricted to the acral region or within psoriatic plaques." Conclusions and Relevance: The achievement of international consensus on the definition and diagnostic criteria for GPP underscores the importance of collaboration, innovative methodology, and expert engagement to address rare diseases. Although further validation is needed, these criteria can serve as a reference point for clinicians, researchers, and patients, which may contribute to more accurate diagnosis and improved management of GPP.

Original languageEnglish
Pages (from-to)758-768
Number of pages11
JournalJAMA dermatology
Volume160
Issue number7
DOIs
Publication statusPublished - 17 Jul 2024

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