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GPP is potentially life\u2010threatening. Recently clarified genetic predisposing factors for GPP suggest that the excessive activation of innate immune pathways in the skin, including of interleukin (IL)\u20101 and IL\u201036 signaling, plays a significant role in the GPP pathogenesis. <jats:italic>IL36RN<\/jats:italic>, <jats:italic>CARD14<\/jats:italic>, <jats:italic>AP1S3<\/jats:italic>, <jats:italic>MPO<\/jats:italic>, <jats:italic>SERPINA3<\/jats:italic>, <jats:italic>BTN3A3<\/jats:italic>, and <jats:italic>MEFV<\/jats:italic> have been identified as GPP\u2010related 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\u201017, and IL\u201023 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\u20101 and IL\u201036 pathways, have been used in clinical practice and investigated as potential future therapies.<\/jats:p>","DOI":"10.1111\/1346-8138.17585","type":"journal-article","created":{"date-parts":[[2024,12,19]],"date-time":"2024-12-19T09:55:41Z","timestamp":1734602141000},"page":"400-407","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Updated genetic background of generalized pustular psoriasis as an autoinflammatory keratinization disease"],"prefix":"10.1111","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5863-9315","authenticated-orcid":false,"given":"Masashi","family":"Akiyama","sequence":"first","affiliation":[{"name":"Department of Dermatology Nagoya University Graduate School of Medicine  Nagoya 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