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In the era of precision medicine in dermatology, GPP stands exemplary for both challenges and chances\u2014while new treatments offer great hope, there is urgent need for better definition and stratification of this severe and heterogeneous disease. Our objective was to systematically review the literature for evidence of efficacy of targeted immunotherapy and their mode of action in the context of clinical phenotype, classification and pathogenesis of adult GPP. Classifying GPP is challenging since clinical criteria for description and diagnosis are not consistent between expert centres. We therefore defined diagnostic feasibility of the reviewed cases by assessing four criteria: compatible clinical history, typical dermatological features and\/or diagnostic histopathology, consistent clinical pictures and the DITRA status. Pathogenesis of GPP is mediated by pathways that partly overlap plaque type psoriasis, with a more pronounced activity of the innate immune system. Both IL\u20101 and IL\u201036 but also IL\u201017 play a major role in disease formation. We ascertained a total of 101 published cases according to our predefined criteria and identified TNF\u2010\u03b1, IL\u201012\/23, IL\u201017 and IL\u20101\u03b2 as targets for immunotherapy for the treatment of GPP. Of those cases, 61% showed complete response and 27% partial response to targeted immunotherapy. Only 12% experienced weak or no response. These data indicate that specific immunotherapy can be used to effectively treat GPP, with most evidence existing for anti\u2010IL\u201017 agents.<\/jats:p>","DOI":"10.1111\/exd.13699","type":"journal-article","created":{"date-parts":[[2018,6,1]],"date-time":"2018-06-01T02:37:43Z","timestamp":1527820663000},"page":"1067-1077","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":82,"title":["Generalized pustular psoriasis \u2013 a model disease for specific targeted immunotherapy, systematic review"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1590-0805","authenticated-orcid":false,"given":"Alexander","family":"Boehner","sequence":"first","affiliation":[{"name":"Department of Dermatology and Allergy Biederstein Technical University of Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander A.","family":"Navarini","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Hospital of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kilian","family":"Eyerich","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy Biederstein Technical University of Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,7,20]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2014.02.006"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013068"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1991.01680080075005"},{"key":"e_1_2_7_5_1","first-page":"669","volume":"16","author":"Augey F.","year":"2006","journal-title":"Eur. 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