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, there is steatosis with occasional lipogranulomas and minimal inflammation. Hepatocytes may contain abundant lipofuscin granules, which are larger and more irregular than normal, as well as bile pigment due to cholestasis.