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

Siew Eng Choon, Peter Anthony Foley, Pravit Asawanonda, Hideki Fujita, Seong Jin Jo, Yu ling Shi, Colin Theng, Azura Mohd Affandi, Chul Hwan Bang, Maria Lorna Frez, Huang Yu Huei, Doanh Le Huu, Tae Gyun Kim, Akimichi Morita, Hazel H. Oon, Pablo Fernández-Peñas, Natta Rajatanavin, Suganthy Robinson, Latha Selvarajah, Tsen Fang Tsai

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7 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1579-1595
Number of pages17
JournalThe Journal of Dermatology
Volume51
Issue number12
DOIs
Publication statusPublished - Dec 2024

Keywords

  • Asia-Pacific
  • consensus
  • Delphi panel
  • generalized pustular psoriasis
  • management

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