Microangiopathy in Ehlers-Danlos syndrome type IV

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.