Article: article from journal or magazin.
Case report (case report): feedback on an observation with a short commentary.
Microangiopathy in Ehlers-Danlos syndrome type IV.
International Journal of Microcirculation, Clinical and Experimental
Publication types: Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
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.
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
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