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| Verfasst von: | Doolan, Brent [VerfasserIn]  |
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| | Lavallee, Mark [VerfasserIn] |
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| | Haußer-Siller, Ingrid [VerfasserIn] |
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| | Pope, F. Michael [VerfasserIn] |
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| | Seneviratne, Suranjith L. [VerfasserIn] |
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| | Winship, Ingrid M. [VerfasserIn] |
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| | Burrows, Nigel P. [VerfasserIn] |
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| Titel: | Dermatologic manifestations and diagnostic assessments of the Ehlers-Danlos syndromes |
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| Titelzusatz: | a clinical review |
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| Verf.angabe: | Brent J. Doolan, Mark Lavallee, Ingrid Hausser, F. Michael Pope, Suranjith L. Seneviratne, Ingrid M. Winship, and Nigel P. Burrows |
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| E-Jahr: | 2023 |
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| Jahr: | September 2023 |
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| Umfang: | 9 S. |
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| Illustrationen: | Illustrationen |
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| Fussnoten: | Online verfügbar: 8. Februar 2023, Artikelversion: 18. August 2023 ; Gesehen am 19.10.2023 |
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| Titel Quelle: | Enthalten in: American Academy of DermatologyJournal of the American Academy of Dermatology |
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| Ort Quelle: | Amsterdam [u.a.] : Elsevier, 1979 |
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| Jahr Quelle: | 2023 |
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| Band/Heft Quelle: | 89(2023), 3 vom: Sept., Seite 551-559 |
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| ISSN Quelle: | 1097-6787 |
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| Abstract: | Background - The Ehlers-Danlos syndromes (EDSs) comprise a group of connective tissue disorders that manifest with skin hyperextensibility, easy bruising, joint hypermobility and fragility of skin, soft tissues, and some organs. A correct assessment of cutaneous features along with the use of adjunct technologies can improve diagnostic accuracy. - Objectives - To systematically review the cutaneous features and adjunct investigations of EDS. - Methods - A search of PubMed and Web of Science for EDS-related cutaneous features and additional investigations was undertaken from publication of the 2017 International Classification of EDS until January 15, 2022. - Results - One-hundred-and-forty studies involved 839 patients with EDS. The EDS female-to-male ratio was 1.36:1 (P < .001). A high prevalence of skin hyperextensibility, bruising, and soft skin were noted. Most patients with vascular Ehlers-Danlos syndrome showed venous visibility, skin fragility, and acrogeria. Classical EDS showed subcutaneous spheroids and molluscoid pseudotumours. In patients that underwent skin biopsies, only 30.3% and 71.4% showed features suggestive of EDS using light microscopy and transmission electron microscopy, respectively. - Limitations - Retrospective study and small cases numbers for some EDS-subtypes. - Conclusions - An accurate clinical diagnosis increases the chances of a molecular diagnosis, particularly for rarer EDS subtypes, whilst decreasing the need for genetic testing where there is a low clinical suspicion for a monogenic EDS-subtype. |
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| DOI: | doi:10.1016/j.jaad.2023.01.034 |
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| URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
kostenfrei: Volltext: https://doi.org/10.1016/j.jaad.2023.01.034 |
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| | kostenfrei: Volltext: https://www.sciencedirect.com/science/article/pii/S0190962223001706 |
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| | DOI: https://doi.org/10.1016/j.jaad.2023.01.034 |
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| Datenträger: | Online-Ressource |
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| Sprache: | eng |
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| Sach-SW: | Ehlers-Danlos syndrome |
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| | heritable connective tissue disorders |
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| | joint hypermobility |
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| | skin fragility |
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| | skin hyperextensibility |
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| K10plus-PPN: | 1866396986 |
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| Verknüpfungen: | → Zeitschrift |
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Dermatologic manifestations and diagnostic assessments of the Ehlers-Danlos syndromes / Doolan, Brent [VerfasserIn]; September 2023 (Online-Ressource)
