Benign Inflammatory Metabolic Disease: Wilson's Disease

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Benign Inflammatory Metabolic Disease: Wilson's Disease

Wilson's disease is caused by a genetic disorder affecting an individual's copper metabolism. A distinguishing characteristic of Wilson's disease is copper accumulation in the liver. Hematoxylin and eosin (H&E) stained tissue slides commonly do not reveal the presence of copper. Copper stains such as rhodanine or rubeanic acid (shown in the image) can be used to positively identify copper in liver biopsy tissue, although quantitative copper studies performed on formalin-fixed, paraffin-embedded (FFPE) tissue are more sensitive and helpful to the pathologist.
Because other liver diseases may also stain positively for copper, pathologists also review the biopsy for the presence of Mallory hyaline which is another distinguishing characteristic of Wilson's disease. The presence of copper and Mallory hyaline are commonly correlated with the clinical history of the patient to positively diagnose the patient with Wilson's disease.

Rubeanic acid stained liver biopsy demonstrating copper deposits.