Microangiopathy in Ehlers-Danlos syndrome type IV.

Details

Serval ID
serval:BIB_E4ACF4E23C06
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Microangiopathy in Ehlers-Danlos syndrome type IV.
Journal
International Journal of Microcirculation, Clinical and Experimental
Author(s)
Superti-Furga A., Saesseli B., Steinmann B., Bollinger A.
ISSN
0167-6865 (Print)
ISSN-L
0167-6865
Publication state
Published
Issued date
1992
Volume
11
Number
3
Pages
241-247
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
Abstract
In two patients with Ehlers-Danlos syndrome type IV, an autosomal dominant disorder characterized by fragility of large vessels, excessive bruising, and deficiency in type III collagen, capillary microscopy was performed at the nailfold. Indocyanine green and Na-fluorescein were used as fluorescent tracers. Both patients exhibited microangiopathy of the skin capillaries with microbleedings, presence of microaneurysms and increased transcapillary diffusion. Microvascular involvement appears to be an additional manifestation of the syndrome.
Keywords
Adult, Aneurysm/etiology, Capillaries/pathology, Collagen/deficiency, Ehlers-Danlos Syndrome/classification, Ehlers-Danlos Syndrome/complications, Female, Fluorescein, Fluoresceins/diagnostic use, Hemorrhage/etiology, Humans, Indocyanine Green/diagnostic use, Male, Microcirculation, Microscopy, Fluorescence, Nails/blood supply, Nails/pathology, Pregnancy, Vascular Diseases/etiology, Vascular Diseases/pathology, Videotape Recording
Pubmed
Web of science
Create date
14/03/2011 16:14
Last modification date
20/08/2019 16:08
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