Kayser-Fleischer ring and associated cataract in Wilson's disease

Abstract

A 22-year-old woman with hepatolenticular degeneration, or Wilson's disease, was note clinically to have Kayser-Fleischer rings and associated cataracts. Histopathologic, histochemical, and electron microscopic study of the cornea showed deposition of copper in the peripheral Descemet's membrane, with excessive accumulation in Hassall-Henel warts. Copper deposits were also noted in the anterior and posterior lens capsule without the presence of degenerative changes in the epithelial or cortical cells of the lens. We propose that cellular activity is required for the depostion of copper material into the thick basement membranes of the cornea and lens, in contrast to the concept of simple diffusion.