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Verfasst von:Doolan, Brent [VerfasserIn]   i
 Lavallee, Mark [VerfasserIn]   i
 Haußer-Siller, Ingrid [VerfasserIn]   i
 Pope, F. Michael [VerfasserIn]   i
 Seneviratne, Suranjith L. [VerfasserIn]   i
 Winship, Ingrid M. [VerfasserIn]   i
 Burrows, Nigel P. [VerfasserIn]   i
Titel:Dermatologic manifestations and diagnostic assessments of the Ehlers-Danlos syndromes
Titelzusatz:a clinical review
Verf.angabe:Brent J. Doolan, Mark Lavallee, Ingrid Hausser, F. Michael Pope, Suranjith L. Seneviratne, Ingrid M. Winship, and Nigel P. Burrows
E-Jahr:2023
Jahr:September 2023
Umfang:9 S.
Illustrationen:Illustrationen
Fussnoten:Online verfügbar: 8. Februar 2023, Artikelversion: 18. August 2023 ; Gesehen am 19.10.2023
Titel Quelle:Enthalten in: American Academy of DermatologyJournal of the American Academy of Dermatology
Ort Quelle:Amsterdam [u.a.] : Elsevier, 1979
Jahr Quelle:2023
Band/Heft Quelle:89(2023), 3 vom: Sept., Seite 551-559
ISSN Quelle:1097-6787
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.
DOI:doi:10.1016/j.jaad.2023.01.034
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
 kostenfrei: Volltext: https://www.sciencedirect.com/science/article/pii/S0190962223001706
 DOI: https://doi.org/10.1016/j.jaad.2023.01.034
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:Ehlers-Danlos syndrome
 heritable connective tissue disorders
 joint hypermobility
 skin fragility
 skin hyperextensibility
K10plus-PPN:1866396986
Verknüpfungen:→ Zeitschrift

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