Nonhomogeneous copper distribution in primary biliary cirrhosis

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Considerable attention has been focused recently upon tissue copper levels in patients with primary biliary cirrhosis (PBC). Elevated hepatic copper levels in PBC were first demonstrated by neutron activation studies of tissue obtained at surgery or postmortem.1 Six patients with PBC had copper levels 30 times normal. These observations were corroborated by others.2–5 There is little information regarding homogeneity of the distribution of copper in the liver, although the available evidence suggests that there are no major regional hepatic differences in PBC.3 A case of PBC has recently been encountered in which regional differences in hepatic copper concentration have been documented. The copper level was elevated in the area of the liver that was most abnormal morphologically and histologically.

Case report

A 44-year-old black woman was examined at the Cleveland Clinic for the first time in September 1977. Her chief complaint was intermittent swelling of both hands for 3 months. She also noted some hard crusted lesions on the volar aspect of the distal and proximal phalanges and on the tips of the fingers. She described a color change associated with a burning sensation when her hands were exposed to cold. She denied esophageal symptoms, pruritus, or constitutional symptoms.

Her height was 164 cm (65.6 inches); weight, 58.5 kg (128.7 pounds); pulse rate, 80 and regular; and blood pressure, 130/78 mm Hg. The general examination revealed a healthy woman. The skin abnormalities were limited to the hands; there were several pitting ulcers of the fingertips and the skin was . . .



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