Cirrhosis in 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 progression from steatosis to chronic hepatitis to cirrhosis (as seen here). The cirrhotic nodules are separated by fibrous septa containing chronic inflammatory infiltrates and abundant ductular structures, some of which may have bile plugs due to cholestasis.