{"title":"Generalized pustular psoriasis: immunological mechanisms, genetics, and emerging therapeutics.","authors":"Chih-Chun Lee, Yu-Huei Huang, Ching-Chi Chi, Wen-Hung Chung, Chun-Bing Chen","doi":"10.1016/j.it.2024.12.001","DOIUrl":null,"url":null,"abstract":"<p><p>Generalized pustular psoriasis (GPP) is a rare human autoinflammatory disorder with life-threatening systemic effects. Keratinocyte-derived interleukin (IL)-36 signaling has been identified as a key mediator of immune response in the skin of affected individuals. Recognition of various mutations along the IL-36 axis and the downstream nuclear transcription factor κB (NF-κB) signaling have established GPP as genetically, immunologically, and histopathologically distinct and amenable to immunomodulation, which is epitomized by the recent success of IL-36 antagonism. This review covers recent discoveries of the genetic and immunological underpinnings of GPP, which have proved fertile ground for improving the quality of care of this clinically challenging and debilitating condition.</p>","PeriodicalId":54412,"journal":{"name":"Trends in Immunology","volume":" ","pages":"74-89"},"PeriodicalIF":13.1000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Trends in Immunology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.it.2024.12.001","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/12/27 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"IMMUNOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Generalized pustular psoriasis (GPP) is a rare human autoinflammatory disorder with life-threatening systemic effects. Keratinocyte-derived interleukin (IL)-36 signaling has been identified as a key mediator of immune response in the skin of affected individuals. Recognition of various mutations along the IL-36 axis and the downstream nuclear transcription factor κB (NF-κB) signaling have established GPP as genetically, immunologically, and histopathologically distinct and amenable to immunomodulation, which is epitomized by the recent success of IL-36 antagonism. This review covers recent discoveries of the genetic and immunological underpinnings of GPP, which have proved fertile ground for improving the quality of care of this clinically challenging and debilitating condition.
期刊介绍：
Trends in Immunology serves as a vital platform for tracking advancements across various areas of immunology, offering concise reviews and hypothesis-driven viewpoints in each issue. With additional sections providing comprehensive coverage, the journal offers a holistic view of immunology. This broad perspective makes it an invaluable resource for researchers, educators, and students, facilitating the connection between basic and clinical immunology. Recognized as one of the top monthly review journals in its field, Trends in Immunology is highly regarded by the scientific community.
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