Wilson Disease

Wilson disease is an uncommon autosomal recessive disorder due to mutations in the ATP7B gene, which disrupt normal hepatic copper transport, leading to copper accumulation in hepatocytes and progressive liver disease. Histologic lesions usually precede clinical disease. In the early stages (as seen here), there is steatosis with occasional lipogranulomas and minimal inflammation. The portal tracts radiate slender fibrous septa. Untreated, these early changes progress to chronic hepatitis, which often resembles chronic viral hepatitis histologically, and cirrhosis may ensue.