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

Choon SE 1 , van de Kerkhof P 2 , Gudjonsson JE 3 , de la Cruz C 4 , Barker J 5 , Morita A 6 Show all authors , Romiti R 7 , Affandi AM 8 , Asawanonda P 9 , Burden AD 10 , Gonzalez C 11 , Marrakchi S 12 , Mowla MR 13 , Okubo Y 14 , Oon HH 15 , Terui T 16 , Tsai TF 17 , Callis-Duffin K 18 , Fujita H 19 , Jo SJ 20 , Merola J 21 , Mrowietz U 22 , Puig L 23 , Thaçi D 24 , Velásquez M 25 , Augustine M 26 , El Sayed M 27 , Navarini AA 28 , Pink A 5 , Prinz J 29 , Turki H 12 , Magalhães R 30 , Capon F 31 , Bachelez H 32 , IPC Pustular Psoriasis Working Group

Affiliations 

  • 1 Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University, Johor Bahru, Malaysia
  • 2 Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands
  • 3 Department of Dermatology and Department of Internal Medicine, University of Michigan, Ann Arbor
  • 4 Clínica Dermacross, Santiago, Chile
  • 5 St John's Institute of Dermatology, King's College London and Guy's and St Thomas's Hospitals, London, United Kingdom
  • 6 Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
  • 7 University of São Paulo, São Paulo, Brazil
  • 8 Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
  • 9 Chulalongkorn University, Bangkok, Thailand
  • 10 University of Glasgow, Glasgow, United Kingdom
  • 11 Private practice, Bogota, Colombia
  • 12 Hedi Chaker Hospital, University of Sfax, Sfax, Tunisia
  • 13 Chittagong Medical College and Comilla Medical College, Chattogram, Bangladesh
  • 14 Tokyo Medical University, Tokyo, Japan
  • 15 National Skin Centre, Lee Kong Chian School of Medicine, Singapore, Singapore
  • 16 Nihon University School of Medicine, Tokyo, Japan
  • 17 National Taiwan University Hospital, Taipei, Taiwan
  • 18 Spencer F Eccles School of Medicine, University of Utah, Salt Lake City
  • 19 Division of Cutaneous Science, Department of Dermatology, Nihon University School of Medicine, Tokyo, Japan
  • 20 Department of Dermatology, Seoul National University College of Medicine, Seoul, Republic of Korea
  • 21 UT Southwestern Medical Center, Dallas, Texas
  • 22 Psoriasis-Center at the Department of Dermatology, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany
  • 23 Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain
  • 24 University of Luebeck, Luebeck, Germany
  • 25 Universidad de Antioquia, Medellin, Colombia
  • 26 University Medical Center of Hamburg, Hamburg, Germany
  • 27 Ain Shams University, Cairo, Egypt
  • 28 Department of Dermatology, University Hospital of Basel, Basel, Switzerland
  • 29 Department of Dermatology and Allergy, University Hospital, Ludwig Maximilian-University of Munich, Munich, Germany
  • 30 Unicamp, São Paulo, Brazil
  • 31 Division of Genetics and Molecular Medicine, King's College, London, United Kingdom
  • 32 Department of Dermatology, Hôpital Saint-Louis APHP, Laboratory of Genetic of Skin Diseases, INSERM U1163, Imagine Institute for Human Genetic Diseases, Paris Cité University, Paris, France
JAMA Dermatol, 2024 May 01.
PMID: 38691347 DOI: 10.1001/jamadermatol.2024.0915

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.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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