Pathology: xanthelasma of PBC

Primary biliary cirrhosis (PBC) affects mostly females and is associated with an elevated anti-mitochondrial antibody (AMA).  This antibody has an excellent positive predictive value.  The only medical treatment for PBC is ursodeoxycholic acid, 15 mg/kg daily, which delays the time to end stage liver disease.

Xanthomas are uncommon in PBC but may be present when the patient’s total cholesterol level is very high; they are, by definition, deposits of cholesterol in the skin.  They may be found periorbitally (xanthelasma) or other places, like extensor surfaces or the palms and soles.

Xanthomas are not the only possible dermatologic finding in PBC, of course.  Much more commonly you will find hyperpigmentation (melanin deposition), pruritus, jaundice, xerosis and onychomycosis.

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