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

Choon SE 1 , Foley PA 2 , Asawanonda P 3 , Fujita H 4 , Jo SJ 5 , Shi YL 6 Show all authors , Theng C 7 , Affandi AM 8 , Bang CH 9 , Frez ML 10 , Huei HY 11 , Le Huu D 12 , Kim TG 13 , Morita A 14 , Oon HH 15 , Fernández-Peñas P 16 , Rajatanavin N 17 , Robinson S 8 , Selvarajah L 18 , Tsai TF 19

Affiliations 

  • 1 Clinical School Johor Bahru, Jeffrey Cheah School of Medicine & Health Sciences, Monash University, Johor Bahru, Malaysia
  • 2 The University of Melbourne, St Vincent's Hospital Melbourne, Fitrozy, Victoria, Australia
  • 3 Division of Dermatology, Chulalongkorn University, Bangkok, Thailand
  • 4 Division of Cutaneous Science, Department of Dermatology, Nihon University School of Medicine, Tokyo, Japan
  • 5 Department of Dermatology, Seoul National University College of Medicine, Seoul, Republic of Korea
  • 6 Department of Dermatology, Shanghai Skin Disease Hospital, School of Medicine, Tongji University, Shanghai, China
  • 7 The Skin Specialists & Laser Clinic, Singapore, Singapore
  • 8 Department of Dermatology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
  • 9 Department of Dermatology, Seoul St. Mary's Hospital, College of Medicine, the Catholic University of Korea, Seoul, Republic of Korea
  • 10 Department of Dermatology, St Luke's Medical Center, Quezon City, Philippines
  • 11 Department of Dermatology, Chang Gung Memorial Hospital, Taipei, Taiwan
  • 12 National Hospital of Dermatology and Venereology, Hanoi, Vietnam
  • 13 Department of Dermatology, Cutaneous Biology Research Institute, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
  • 14 Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, 467-8601, Japan
  • 15 National Skin Centre and Skin Research Institute of Singapore (SRIS), Singapore, Singapore
  • 16 Department of Dermatology, Westmead Hospital, Westmead, New South Wales, Australia
  • 17 Phototherapy Unit, Division of Dermatology, Department of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
  • 18 Department of Dermatology, Sultan Ismail Hospital, Johor Bahru, Malaysia
  • 19 Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan
J Dermatol, 2024 Dec;51(12):1579-1595.
PMID: 39390737 DOI: 10.1111/1346-8138.17471

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.

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

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