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May 1976

Pigmented Corneal Rings in a Patient With Primary Biliary Cirrhosis

Author Affiliations

Mayo Clinic Rochester, MN 55901

Arch Neurol. 1976;33(5):372. doi:10.1001/archneur.1976.00500050058013

To the Editor.—  Since the presence of Kayser-Fleischer rings has been deemed pathognomic of Wilson disease (hepatolenticular degeneration), I would like to call your attention to an exception to that rule.1A patient with primary biliary cirrhosis (PBC) was found by slit-lamp examination to have pigmented corneal rings that were similar to Kayser-Fleischer rings. Although the exact nature of these corneal rings is unknown, this patient with PBC had increased hepatic, serum, and urine copper levels. Wilson disease was excluded, despite the presence of these pigmented corneal rings, by the serum ceruloplasmin and copper concentrations, in conjunction with the lack of neurologic disease (despite the advanced hepatic dysfunction) and the typical clinical picture of PBC.Apparently, not all patients with pigmented corneal rings have Wilson disease.