{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T21:07:29Z","timestamp":1790716049341,"version":"4.1.0"},"reference-count":48,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,2,4]],"date-time":"2023-02-04T00:00:00Z","timestamp":1675468800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008349","name":"Boehringer Ingelheim","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008349","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Generalized pustular psoriasis (GPP) is a rare and highly heterogeneous skin disease, characterized by flares of neutrophilic pustules and erythema. As a rare disease with few clinical studies and no standardized management approaches, there is a paucity of knowledge regarding GPP.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Conduct a Delphi panel study to identify current evidence and gain advanced insights into GPP.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A systematic literature review was used to identify published literature and develop statements categorized into four key domains: clinical course and flare definition; diagnosis; treatment goals; and holistic management. Statements were rated on a Likert scale by a panel of dermatologists in two rounds of online questionnaires; the threshold for consensus was agreement by \u226580%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Twenty\u2010one panellists reached consensus on 70.9%, 61.8%, 100.0% and 81.8% of statements in the \u2018clinical course and flare definition\u2019, \u2018diagnosis\u2019, \u2018treatment goals\u2019 and \u2018holistic management of GPP\u2019 domains, respectively. There was clear consensus on GPP being phenotypically, genetically and immunologically distinct from plaque psoriasis. Clinical course is highly variable, with an extensive range of complications. Clinical and histologic features supporting GPP diagnosis reached high levels of agreement, and although laboratory evaluations were considered helpful for diagnosis and monitoring disease severity, there was uncertainty around the value of individual tests. All acute and long\u2010term treatment goals reached consensus, including rapid and sustained clearance of pustules, erythema, scaling and crust, clearance of skin lesions and prevention of new flares. Potential triggers, associated comorbidities and differential diagnoses achieved low rates of consensus, indicating that further evidence is needed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Global consensus between dermatologists was reached on clinically meaningful goals for GPP treatment, on key features of GPP flares and on approaches for assessing disease severity and multidisciplinary management of patients. On this basis, we present a management algorithm for patients with GPP for use in clinical practice.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/jdv.18851","type":"journal-article","created":{"date-parts":[[2023,1,6]],"date-time":"2023-01-06T05:54:55Z","timestamp":1672984495000},"page":"737-752","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":84,"title":["Generalized pustular psoriasis: A global Delphi consensus on clinical course, diagnosis, treatment goals and disease management"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6083-0952","authenticated-orcid":false,"given":"Llu\u00eds","family":"Puig","sequence":"first","affiliation":[{"name":"Department of Dermatology Hospital de la Santa Creu i Sant Pau  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7796-5746","authenticated-orcid":false,"given":"Siew Eng","family":"Choon","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hospital Sultanah Aminah, and Clinical School Johor Bahru, Monash University Malaysia  Johor Bahru Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice B.","family":"Gottlieb","sequence":"additional","affiliation":[{"name":"Icahn School of Medicine at Mount Sinai  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Slaheddine","family":"Marrakchi","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hedi Chaker Hospital, University of Sfax  Sfax Tunisia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg C.","family":"Prinz","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergy Ludwig\u2010Maximilian\u2010University Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0165-3831","authenticated-orcid":false,"given":"Ricardo","family":"Romiti","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of S\u00e3o Paulo  S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yayoi","family":"Tada","sequence":"additional","affiliation":[{"name":"Department of Dermatology Teikyo University School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorothea","family":"von Bredow","sequence":"additional","affiliation":[{"name":"IQVIA Commercial GmbH &amp; Co.  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