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The inflammatory nature of GPP has potential for severe multisystem complications including high\u2010output cardiac failure, infections, digestive system issues, and disfiguring or lethal acute flare episodes. The disease tends to have higher prevalence in females and Asians. The IL\u20101\/IL\u201036 inflammatory pathway is a critical facet of GPP's pathology. Genetic mutations that are associated with GPP include modifications of Interleukin 36 Receptor Antagonist (\n                    <jats:italic>IL36RN<\/jats:italic>\n                    ), Caspase Recruitment Domain Family Member 14 (\n                    <jats:italic>CARD14<\/jats:italic>\n                    ), Adaptor Related Protein Complex 1 Subunit Sigma 3 (\n                    <jats:italic>AP1S3<\/jats:italic>\n                    ), Myeloperoxidase (\n                    <jats:italic>MPO<\/jats:italic>\n                    ) and Serpin Peptidase Inhibitor Clade A Member 3 (\n                    <jats:italic>SERPINA3)<\/jats:italic>\n                    genes. Treatment guidelines for GPP are not well\u2010entrenched. Currently, only one GPP\u2010specific treatment, the interleukin\u201036 receptor antagonist (IL\u201036Ra) spesolimab, has been approved for use in the United States. Additional anti\u2010IL\u201036 pathway therapies are currently being developed. Other treatment options include other biologic therapies such as IL\u201017 inhibitors, IL\u201023 inhibitors and TNF\u03b1 inhibitors. Non\u2010biologic therapeutic options include retinoids, cyclosporine and methotrexate. Treatment options differ throughout the world; most countries utilize retinoids, cyclosporine and methotrexate as first\u2010line non\u2010biologic options. China and United Kingdom have no GPP\u2010specific biologic therapies approved for use, while several biologic therapies are approved for use in Japan. This review aims to serve as an update on the current global management of GPP while also including relevant aspects of disease pathogenesis, diagnosis, clinical presentation, histopathology, aetiology and epidemiology.\n                  <\/jats:p>","DOI":"10.1111\/exd.14765","type":"journal-article","created":{"date-parts":[[2023,2,13]],"date-time":"2023-02-13T08:35:10Z","timestamp":1676277310000},"page":"1204-1218","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":70,"title":["Current management of generalized pustular psoriasis"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2092-9469","authenticated-orcid":false,"given":"Nilesh","family":"Kodali","sequence":"first","affiliation":[{"name":"Department of Education Rutgers New Jersey Medical School  Newark New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4995-031X","authenticated-orcid":false,"given":"Isabella","family":"Blanchard","sequence":"additional","affiliation":[{"name":"Department of Education Rutgers New Jersey Medical School  Newark New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6443-6079","authenticated-orcid":false,"given":"Sruthi","family":"Kunamneni","sequence":"additional","affiliation":[{"name":"Department of Education Rutgers New Jersey Medical School  Newark New Jersey USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark G.","family":"Lebwohl","sequence":"additional","affiliation":[{"name":"Dermatology The Icahn School of Medicine at Mount Sinai  New York City New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,3]]},"reference":[{"key":"e_1_2_19_2_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.788940"},{"key":"e_1_2_19_3_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-3388"},{"key":"e_1_2_19_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(07)61128-3"},{"key":"e_1_2_19_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.09.049"},{"key":"e_1_2_19_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.12070"},{"key":"e_1_2_19_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.14523"},{"key":"e_1_2_19_8_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2019.1708193"},{"key":"e_1_2_19_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2020.07.001"},{"key":"e_1_2_19_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13555-021-00661-2"},{"key":"e_1_2_19_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13555-022-00835-6"},{"key":"e_1_2_19_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18720"},{"issue":"6","key":"e_1_2_19_13_1","first-page":"669","article-title":"Generalized pustular psoriasis (Zumbusch): a French epidemiological survey","volume":"16","author":"Augey F","year":"2006","journal-title":"Eur J Dermatol"},{"key":"e_1_2_19_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdin.2021.12.001"},{"key":"e_1_2_19_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00403-022-02429-w"},{"key":"e_1_2_19_16_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555766871"},{"key":"e_1_2_19_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.12863"},{"key":"e_1_2_19_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2021.06.008"},{"key":"e_1_2_19_19_1","unstructured":"Vmx A. 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