{"id":11061,"date":"2024-12-19T11:45:00","date_gmt":"2024-12-19T10:45:00","guid":{"rendered":"https:\/\/www.adacyte.com\/?post_type=scientific-corner&#038;p=11061"},"modified":"2025-08-08T11:50:40","modified_gmt":"2025-08-08T09:50:40","slug":"updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease","status":"publish","type":"scientific-corner","link":"https:\/\/www.adacyte.com\/fr\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/","title":{"rendered":"Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease"},"content":{"rendered":"<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p><a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/?sort=date&amp;term=Akiyama+M&amp;cauthor_id=39698752\">Masashi Akiyama<\/a><sup>\u00a0<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/39698752\/#full-view-affiliation-1\">1<\/a><\/sup><\/p>\n<\/div>\n\n<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p>J Dermatol.\u00a02025 Mar;52(3):400-407.\u00a0doi: 10.1111\/1346-8138.17585.\u00a0Epub 2024 Dec 19.<\/p>\n<\/div>\n\n<div class=\"post-content max-w-987 mx-auto px-20 lg:px-0\">\n<p>Generalized pustular psoriasis (GPP) is a severe autoinflammatory keratinization disease (AiKD) characterized by acute flares of widespread sterile pustules and high fever. GPP is potentially life-threatening. Recently clarified genetic predisposing factors for GPP suggest that the excessive activation of innate immune pathways in the skin, including of interleukin (IL)-1 and IL-36 signaling, plays a significant role in the GPP pathogenesis. IL36RN, CARD14, AP1S3, MPO, SERPINA3, BTN3A3, and MEFV have been identified as GPP-related genes. The pathogenesis of GPP provoked by variants in these seven genes is tightly associated with the excessive activation of innate immune pathways and the resulting autoinflammation in the skin. Various biologics, including inhibitors for the tumor necrosis factor, IL-17, and IL-23 pathways, are used as treatments for GPP. The new understanding of the genetic background of GPP, mentioned above, indicates that the genetic predisposing factors are predominantly related to the excessive activation of innate immunity and autoinflammation. In this context, inhibitors of inflammatory signaling, including of the IL-1 and IL-36 pathways, have been used in clinical practice and investigated as potential future therapies.<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>Masashi Akiyama\u00a01 J Dermatol.\u00a02025 Mar;52(3):400-407.\u00a0doi: 10.1111\/1346-8138.17585.\u00a0Epub 2024 Dec 19. Generalized pustular psoriasis (GPP) is a severe autoinflammatory keratinization disease (AiKD) characterized by acute flares of widespread sterile pustules and high fever. GPP is potentially life-threatening. Recently clarified genetic predisposing factors for GPP suggest that the excessive activation of innate immune pathways in the skin, including [&hellip;]<\/p>\n","protected":false},"template":"","scientific_tags":[608,880,712,711,882,73,348,424,575,658,130,808,883,581,881],"class_list":["post-11061","scientific-corner","type-scientific-corner","status-publish","hentry","scientific_tags-activated-neutrophils","scientific_tags-ap1s3","scientific_tags-autoinflammatory-keratinization-disease","scientific_tags-autoinflammmatory","scientific_tags-btn3a3","scientific_tags-card14","scientific_tags-generalized-pustular-psoriasis","scientific_tags-gma","scientific_tags-il-1b","scientific_tags-il-36","scientific_tags-il36rn","scientific_tags-innate-immunity","scientific_tags-mefv","scientific_tags-mpo","scientific_tags-serpina3"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v22.9 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease - Adacyte<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease - Adacyte\" \/>\n<meta property=\"og:description\" content=\"Masashi Akiyama\u00a01 J Dermatol.\u00a02025 Mar;52(3):400-407.\u00a0doi: 10.1111\/1346-8138.17585.\u00a0Epub 2024 Dec 19. Generalized pustular psoriasis (GPP) is a severe autoinflammatory keratinization disease (AiKD) characterized by acute flares of widespread sterile pustules and high fever. GPP is potentially life-threatening. Recently clarified genetic predisposing factors for GPP suggest that the excessive activation of innate immune pathways in the skin, including [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/\" \/>\n<meta property=\"og:site_name\" content=\"Adacyte\" \/>\n<meta property=\"article:modified_time\" content=\"2025-08-08T09:50:40+00:00\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Dur\u00e9e de lecture estim\u00e9e\" \/>\n\t<meta name=\"twitter:data1\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/\",\"url\":\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/\",\"name\":\"Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease - Adacyte\",\"isPartOf\":{\"@id\":\"https:\/\/www.adacyte.com\/#website\"},\"datePublished\":\"2024-12-19T10:45:00+00:00\",\"dateModified\":\"2025-08-08T09:50:40+00:00\",\"inLanguage\":\"fr-FR\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/www.adacyte.com\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/\"]}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/www.adacyte.com\/#website\",\"url\":\"https:\/\/www.adacyte.com\/\",\"name\":\"Adacyte\",\"description\":\"Adacyte Therapeutics\",\"publisher\":{\"@id\":\"https:\/\/www.adacyte.com\/#organization\"},\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/www.adacyte.com\/?s={search_term_string}\"},\"query-input\":\"required name=search_term_string\"}],\"inLanguage\":\"fr-FR\"},{\"@type\":\"Organization\",\"@id\":\"https:\/\/www.adacyte.com\/#organization\",\"name\":\"Adacyte\",\"url\":\"https:\/\/www.adacyte.com\/\",\"logo\":{\"@type\":\"ImageObject\",\"inLanguage\":\"fr-FR\",\"@id\":\"https:\/\/www.adacyte.com\/#\/schema\/logo\/image\/\",\"url\":\"https:\/\/www.adacyte.com\/wp-content\/uploads\/2021\/01\/logo-adacyte.svg\",\"contentUrl\":\"https:\/\/www.adacyte.com\/wp-content\/uploads\/2021\/01\/logo-adacyte.svg\",\"width\":165,\"height\":46,\"caption\":\"Adacyte\"},\"image\":{\"@id\":\"https:\/\/www.adacyte.com\/#\/schema\/logo\/image\/\"}}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease - Adacyte","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/www.adacyte.com\/professional\/scientific-corner\/updated-genetic-background-of-generalized-pustular-psoriasis-as-an-autoinflammatory-keratinization-disease\/","og_locale":"fr_FR","og_type":"article","og_title":"Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease - Adacyte","og_description":"Masashi Akiyama\u00a01 J Dermatol.\u00a02025 Mar;52(3):400-407.\u00a0doi: 10.1111\/1346-8138.17585.\u00a0Epub 2024 Dec 19. Generalized pustular psoriasis (GPP) is a severe autoinflammatory keratinization disease (AiKD) characterized by acute flares of widespread sterile pustules and high fever. GPP is potentially life-threatening. 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