Ultrastructural variation in granular dystrophy of the cornea

Abstract

Corneal deposits of granular dystrophy were studies in six corneas from five patients. Two additional corneas from two patients with lattice dystrophy were used for comparison. The diagnoses were confirmed by light and electron microscopy. Three types of ultrastructural features were distinguished in the electron-dense rod-shaped deposits of granular dystrophy, i.e., Type 1 with homogenous structure, Type 2 with fibrillar constituents, and Type 3 with a moth-eaten or sponge-like appearance. In polarization microscopy of Congo-red stained sections, the Type 2 tended to show an increased birefringence without green dichroism, while the other types were less birefringent than the stroma. Three different components were found immediately around the deposits, i.e., 1. normal collagen fibrils, 2. finely filamentous substance, and 3. areas of thin fibrils (70-100 A in diameter). While the second component appeared non-specific, the third component was found only in two corneas, superimposed on the lesions of granular dystrophy, and showed light and electron microscopic characteristics of the lesions of amyloid as well as of lattice dystrophy.