Groenouw's macular dystrophy: Histochemistry and ultrastructure of the cornea

Abstract

Corneal macular dystrophy - Groenouw Type II - is related to a local deficiency of the hydrolytic enzyme α-Galactosidase. The disease originates from the stromal keratocytes and affects progressively Bowman's and Descemet's membranes.Examining such an affected cornea by means of light microscopy, histochemistry and electron microscopy, these findings were confirmed. In addition, we observed in the stroma vacuolated histiocytes with proteolitic activity invading the Bowman's and Descemet's membranes. Although obvious abnormalities of the corneal structures were disclosed, the endothelium appeared still to be normal.