[Vascular lesions of Fabry's disease. Optical, histochemical and ultrastructural studies]

Abstract

On the basis of a new case of Fabry's disease and the various cases published in the literature, the authors analyse the vascular lesions of this angiodysplasia related to an error in lipid metabolism. They emphasise the involvement of lymph vessels (in particular those of the small bowel), and the ultrastructural characteristics and the mode of formation of deposits within the endothelial cells of the vascular walls.