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Skin disorders are also common clinical features in <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s and may be among the presenting manifestations. Recognition of specific <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>\u2010associated skin conditions in combination with other clinical features as described in the currently used warning signs could raise suspicion of an underlying <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>. We aimed to provide a systematically obtained overview of skin disorders and their prevalence in <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s. Secondary, the prevalence of <jats:italic>Staphylococcus<\/jats:italic> (<jats:italic>S<\/jats:italic>.) <jats:italic>aureus<\/jats:italic>\u2013associated skin disorders and atopy was reviewed, as these are the most prominent skin features in <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s. A systematic search was performed in <jats:styled-content style=\"fixed-case\">EMBASE<\/jats:styled-content>,<jats:styled-content style=\"fixed-case\"> MEDLINE<\/jats:styled-content>, Web of Science, Cochrane, and Google Scholar (up to May 9, 2018). All original observational and experimental human studies that address the presence of skin disorders in <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s were selected. We rated study quality using the Institute of Health Economics Quality Appraisal Checklist for Case Series Studies. Sixty\u2010seven articles (5030 patients) were included. Study quality ranged from 18.2% to 88.5%. A broad spectrum of skin disorders was reported in 30 <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s, mostly in single studies with a low number of included patients. An overview of associated <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s per skin disorder was generated. Data on <jats:italic>S.\u00a0aureus<\/jats:italic>\u2010associated skin disorders and atopy in <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s were limited. In conclusion, skin disorders are prominent features in <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s. Through clustering of <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s per skin disorder, we provide a support tool to use in clinical practice that should raise awareness of <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s based on presenting skin manifestations.<\/jats:p>","DOI":"10.1111\/all.13681","type":"journal-article","created":{"date-parts":[[2018,11,27]],"date-time":"2018-11-27T17:13:54Z","timestamp":1543338834000},"page":"464-482","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Skin disorders are prominent features in primary immunodeficiency diseases: A systematic overview of current data"],"prefix":"10.1111","volume":"74","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5080-1453","authenticated-orcid":false,"given":"Jill","family":"de Wit","sequence":"first","affiliation":[{"name":"Department of Dermatology Erasmus MC University Medical Center Rotterdam the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romke J. 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