We describe a 33-yr-old pregnant woman in whom a primary biliary cirrh
osis-like syndrome developed after 2 wk of chlorpromazine therapy. The
clinical course was characterized by severe jaundice lasting 22 mo, i
ntense pruritus, fever, steatorrhea, high alkaline phosphatase levels
and hypercholesterolemia. Jaundice resolved with initiation of ursodeo
xycholic acid therapy, but subclinical cholestasis and low-level infla
mmatory activity persisted and ultimately evolved into biliary cirrhos
is. The pathological substrate of this severe and prolonged cholestati
c reaction was found to be the vanishing bile duct syndrome with a mar
ked transient pseudoxanthomatosis.