{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T20:24:05Z","timestamp":1791923045318,"version":"4.3.4"},"reference-count":65,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2017,8,29]],"date-time":"2017-08-29T00:00:00Z","timestamp":1503964800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"European Association of Dermatology and Venereology (EADV)"},{"name":"Bruno Bloch and Promedica Foundation"},{"DOI":"10.13039\/100013126","name":"The Psoriasis Association","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100013126","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pustular psoriasis (<jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content>) is a group of inflammatory skin conditions characterized by infiltration of neutrophil granulocytes in the epidermis to such an extent that clinically visible sterile pustules develop. Because of clinical co\u2010incidence, <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content> is currently grouped with psoriasis vulgaris (<jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>). However, <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> are phenotypically different, respond differently to treatments and seem to be distinct on the genetic level. In contrast to <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>, the phenotypes of <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content> are not well defined. Descriptions of each form of <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content> are discordant among standard dermatology textbooks [<jats:italic>Saurat Dermatologie<\/jats:italic> 2016, <jats:italic>Rook's Dermatology<\/jats:italic> 2016, <jats:italic>Fitzpatrick's<\/jats:italic> 2012 and <jats:italic>Braun\u2010Falco<\/jats:italic> 2012], encumbering the collection of phenotypically well\u2010matched groups of patients as well as clinical trials. The European Rare and Severe Psoriasis Expert Network (<jats:styled-content style=\"fixed-case\">ERASPEN<\/jats:styled-content>) was founded to define consensus criteria for diagnosis, deeply phenotype large groups of <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content> patients, analyse the genetics and pathophysiology and prepare for prospective clinical trials. This work reviews historical aspects of these conditions, new genetic findings and presents our initial considerations on the phenotypes of <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content> and a consensus classification of clinical phenotypes that will be used as a baseline for further, prospective studies of <jats:styled-content style=\"fixed-case\">PP<\/jats:styled-content>. Generalized pustular psoriasis (<jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content>) is defined as primary, sterile, macroscopically visible pustules on non\u2010acral skin (excluding cases where pustulation is restricted to psoriatic plaques). <jats:styled-content style=\"fixed-case\">GPP<\/jats:styled-content> can occur with or without systemic inflammation, with or without <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content> and can either be a relapsing (&gt;1 episode) or persistent (&gt;3\u00a0months) condition. Acrodermatitis continua of Hallopeau (<jats:styled-content style=\"fixed-case\">ACH<\/jats:styled-content>) is characterized by primary, persistent (&gt;3\u00a0months), sterile, macroscopically visible pustules affecting the nail apparatus. Palmoplantar pustulosis (<jats:styled-content style=\"fixed-case\">PPP<\/jats:styled-content>) has primary, persistent (&gt;3\u00a0months), sterile, macroscopically visible pustules on palms and\/or soles and can occur with or without <jats:styled-content style=\"fixed-case\">PV<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/jdv.14386","type":"journal-article","created":{"date-parts":[[2017,6,6]],"date-time":"2017-06-06T08:02:58Z","timestamp":1496736178000},"page":"1792-1799","source":"Crossref","is-referenced-by-count":414,"title":["European consensus statement on phenotypes of pustular psoriasis"],"prefix":"10.1111","volume":"31","author":[{"given":"A.A.","family":"Navarini","sequence":"first","affiliation":[{"name":"Department of Dermatology University Hospital of Zurich Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.D.","family":"Burden","sequence":"additional","affiliation":[{"name":"Institute of Infection Inflammation and Immunity University of Glasgow Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Capon","sequence":"additional","affiliation":[{"name":"Division of Genetics and Molecular Medicine King's College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U.","family":"Mrowietz","sequence":"additional","affiliation":[{"name":"Psoriasis Center at the Department of Dermatology University Medical Center, Schleswig\u2010Holstein, Campus Kiel Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6083-0952","authenticated-orcid":false,"given":"L.","family":"Puig","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hospital de la Santa Creu i Sant Pau Universitat Aut\u00f2noma de Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"K\u00f6ks","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venerology Tartu University Hospital Tartu Estonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Kingo","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Venerology Tartu University Hospital Tartu Estonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Smith","sequence":"additional","affiliation":[{"name":"Division of Genetics and Molecular Medicine King's College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.N.","family":"Barker","sequence":"additional","affiliation":[{"name":"Division of Genetics and Molecular Medicine King's College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the ERASPEN Network","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,8,29]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000372-200208000-00016"},{"key":"e_1_2_8_3_1","first-page":"108","article-title":"Pustular psoriasis of the extremities","volume":"86","author":"Barber HW","year":"1936","journal-title":"Guy's Hospital Rep"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2011.07.022"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1013068"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2014.02.006"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2014.09.043"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2012.490"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2015.186"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.230"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.214"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00403-015-1611-x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.361"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2013.44"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2014.3848"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12631"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2014.04.005"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2015.288"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2012.03.013"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01910970"},{"key":"e_1_2_8_21_1","first-page":"371","article-title":"Generalized acute pustulosis. 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