Polymorphic amyloid degeneration of the cornea. A clinical and histopathologic study.
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.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000682:Amyloid; D003315:Cornea / Q000473:pathology* / Q000648:ultrastructure; D003317:Corneal Dystrophies, Hereditary / Q000175:diagnosis; D003318:Corneal Opacity / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010375:Pedigree
D000682:Amyloid