Pigmented Corneal Rings in Non-Wilsonian Liver Disease

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Abstract
Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemet''s membrane that were deemed pathognomonic of Wilson''s disease. Exceptions in 4 patients with non-Wilsonian liver disease were observed. Three patients had primary biliary cirrhosis and 1 patient had chronic aggressive hepatitis with cirrhosis. Pigmented corneal rings were seen by slit-lamp examination. Hepatic, serum and urinary Cu and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. 64Cu or 67Cu studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilson''s disease in that all 3 patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented Cu incorporation into ceruloplasmin. In 1 patient in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.