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It is known that particular caspase recruitment domain family member 14 (<jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic>) gene mutations are associated with familial <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content> and certain forms of psoriasis. Additionally, few data are available about the role of <jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic> gene variants in sporadic <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content>. The clinical picture is variable for the different types of <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content>, therefore choosing the adequate treatment is often difficult, furthermore there are no specific guidelines for therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>Our aim was to survey the efficacy of the applied therapies and to screen the <jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic> gene variants in our <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content> patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this retrospective study, patients diagnosed with <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content> between 2006 and 2016 at our clinic were involved. Besides the follow\u2010up study of the treatments, the genetic analysis of <jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic> gene was performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We analysed 19 patients, among whom 17 were diagnosed with type I, one with type <jats:styled-content style=\"fixed-case\">III<\/jats:styled-content>, and one with type V <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content>. The majority of the patients were successfully treated with acitretin in combination with systemic corticosteroids, and the remaining patients were treated with other systemic therapies with diverse effects. The genetic screening of <jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic> gene revealed two previously described mutations (rs114688446, rs117918077) and six polymorphisms (rs28674001, rs2066964, rs34367357, rs11653893, rs11652075, rs2289541). Ten of 19 patients carried different <jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic> genetic variants either alone or in combination.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Based on our experience, we propose that acitretin and an initial combination of short\u2010term systemic corticosteroid therapy could be a successful treatment option for <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content>. Although we identified several <jats:italic><jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14<\/jats:italic> variants in almost half of our cases, we did not find a correlation between the therapeutic response and the genetic background. Our data support the previous observation that <jats:styled-content style=\"fixed-case\">CARD<\/jats:styled-content>14 genetic variants are not specific to <jats:styled-content style=\"fixed-case\">PRP<\/jats:styled-content>, although they may indicate chronic inflammation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.15455","type":"journal-article","created":{"date-parts":[[2019,1,30]],"date-time":"2019-01-30T05:30:34Z","timestamp":1548826234000},"page":"944-949","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["The management and genetic background of pityriasis rubra pilaris: a single\u2010centre experience"],"prefix":"10.1111","volume":"33","author":[{"given":"B.","family":"G\u00e1l","sequence":"first","affiliation":[{"name":"Department of Dermatology and Allergology University of Szeged Szeged 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