Primary amyloidosis presenting as “sicca complex” and severe intrahepatic cholestasis

An elderly man presented with the sicca complex of dry eyes with xerostomia and severe intrahepatic cholestasis simulating bile duct obstruction. No evidence of an underlying connective tissue disorder, chronic illness, scurvy or myeloma was found. Postmortem examination disclosed generalized amyloidosis with extensive involvement of the salivary glands and liver. This unusual combination of rare manifestations of primary amyloidosis once again emphasizes the multiple clinical guises of the disease.