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Psoriasis can present in several clinical forms. Of these, generalized pustular psoriasis is an acute, severe form, associated with increased morbidity and mortality. Unlike the more common plaque psoriasis, which is thought to feature dysregulation of the adaptive immune system, generalized pustular psoriasis reflects heightened autoinflammatory responses. Recent advances in genetic and immunological studies highlight a key role of the IL\u201036 immune axis in the pathogenesis of generalized pustular psoriasis. In this article, we review the psoriatic subtypes and discuss diagnostic criteria of generalized pustular psoriasis, discuss several newly identified genetic variants associated with pustular disease in the skin, and discuss how these mutations shed light on pustular disease mechanisms. Furthermore, we gather insights from recent transcriptomic studies that similarly implicate a pathogenic role of the IL\u201036 immune axis in generalized pustular psoriasis.<\/jats:p>","DOI":"10.1111\/exd.14768","type":"journal-article","created":{"date-parts":[[2023,2,13]],"date-time":"2023-02-13T08:35:55Z","timestamp":1676277355000},"page":"1194-1203","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["Pathophysiology of generalized pustular psoriasis"],"prefix":"10.1111","volume":"32","author":[{"given":"Kelly Z.","family":"Young","sequence":"first","affiliation":[{"name":"University of Michigan Medical School  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mrinal K.","family":"Sarkar","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of Michigan  Ann Arbor Michigan 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