Secondary biliary cirrhosis in a patient with histiocytosis X.

Abstract

Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesions appear is unknown. Cholestasis results from phenomena of sclerosant colangitis, which affects the intrahepatic ducts, or from proliferation of histiocytic cells in the periportal areas. Some patients develop biliar cyrrhosis. The authors present the clinical case of a 62-year-old female patient, hospitalized for chronic cholestasis, diabetes and gallstone in the main bile duct. She had metastatic lesions of the hypophysis and bones, the biopsies of which revealed infiltration by histiocytic cells. Endoscopic Retrograde Colangiopancreatography (E.R.C.P.) revealed dilatation of intrahepatic bile ducts and stenosis of left hepatic duct. A histological study of the hepatic biopsy showed chronic cholestasis and areas of fibrosis, without infiltration by histiocytic granulomas, which were observed in the medular biopsy and in the thyroid nodule cytology.