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• W2903532032 endingPage "482" @default.
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• W2903532032 abstract "Abstract Primary immunodeficiency diseases ( PID s) are characterized by an increased risk of infections, autoimmunity, autoinflammation, malignancy, and allergic disorders. Skin disorders are also common clinical features in PID s and may be among the presenting manifestations. Recognition of specific PID ‐associated skin conditions in combination with other clinical features as described in the currently used warning signs could raise suspicion of an underlying PID . We aimed to provide a systematically obtained overview of skin disorders and their prevalence in PID s. Secondary, the prevalence of Staphylococcus ( S .) aureus –associated skin disorders and atopy was reviewed, as these are the most prominent skin features in PID s. A systematic search was performed in EMBASE , MEDLINE , 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 PID s were selected. We rated study quality using the Institute of Health Economics Quality Appraisal Checklist for Case Series Studies. Sixty‐seven articles (5030 patients) were included. Study quality ranged from 18.2% to 88.5%. A broad spectrum of skin disorders was reported in 30 PID s, mostly in single studies with a low number of included patients. An overview of associated PID s per skin disorder was generated. Data on S. aureus ‐associated skin disorders and atopy in PID s were limited. In conclusion, skin disorders are prominent features in PID s. Through clustering of PID s per skin disorder, we provide a support tool to use in clinical practice that should raise awareness of PID s based on presenting skin manifestations." @default.
• W2903532032 created "2018-12-11" @default.
• W2903532032 creator A5026793311 @default.
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• W2903532032 creator A5057358627 @default.
• W2903532032 creator A5090630130 @default.
• W2903532032 date "2018-12-27" @default.
• W2903532032 modified "2023-09-25" @default.
• W2903532032 title "Skin disorders are prominent features in primary immunodeficiency diseases: A systematic overview of current data" @default.
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• W2903532032 doi "https://doi.org/10.1111/all.13681" @default.
• W2903532032 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30480813" @default.
• W2903532032 hasPublicationYear "2018" @default.
• W2903532032 type Work @default.

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