Familial Expression of Spontaneous Cervical Artery Dissections and Ehlers-Danlos Syndrome Hypermobile Type

AuthorTrinh Hermanns-Leen
AuthorDaniel Manicourten
AuthorGerald E. Pierarden
Issued Date2014-12-01en
AbstractIntroduction: Spontaneous cervical artery dissection (SCAD) and Ehlers-Danlos syndrome hypermobile type (EDSH) represent two heritable clinical connective tissue disorders, which show some abnormal ultrastructural features in the dermis. Genetic testing for most of those patients is not available at this time. Case Presentation: We present a woman with SCAD and her five close relatives showing obvious or suggestive signs of EDSH. In this family, the ultrastructural aspects of both diseases were closely related. Conclusions: Alterations were present in both the aspect and spacing of collagen fibrils and in the shape of elastic fibers. The present familial combination of SCAD and EDSH, with almost similar ultrastructural features suggests a possible connection between both conditions.en
DOIhttps://doi.org/10.17795/jssc27023en
KeywordSkinen
KeywordCollagenen
KeywordTissuesen
KeywordBlood Vesselsen
KeywordMicroscopyen
KeywordElectronen
PublisherBrieflandsen
TitleFamilial Expression of Spontaneous Cervical Artery Dissections and Ehlers-Danlos Syndrome Hypermobile Typeen
TypeCase Reporten

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