Polymorphic amyloid degeneration of the cornea. A clinical and histopathologic study
M. J. Mannis, J. H. Krachmer, M. M. Rodrigues and G. J. Pardos
The occurrence of polymorphic punctate and filamentous opacities in the
axial cornea of patients in the fourth decade of life and older represents
a distinct clinical entity. The glass-like deposits are usually in the
deeper layers of the cornea and are associated with normal intervening
stroma. Although it is not a cause of visual dysfunction, this disorder may
be confused with lattice corneal dystrophy or with the corneal deposits in
some dysproteinemias. Common clinical findings were found in 14 patients,
and these findings were contrasted with the findings in lattice corneal
dystrophy. Family studies failed to demonstrate heritability, and lesions
were found in older patients only. Histopathologic examination identified
the lesions as amyloid. The findings suggest that his disorder should be
classified as a corneal degeneration. "Polymorphic amyloid degeneration" is
a descriptive for this condition.