Cholestatic liver disease in a 20-yr-old woman with histiocytosis X.

A 20-yr-old woman with known diabetes insipidus complained of high fever and jaundice. Liver specimens biopsied under peritoneoscopy showed proliferating histiocytic or foamy cells in periportal areas. Immunocytochemical investigation demonstrated the presence of S-100 protein, but the absence of lysozyme in their histiocytic cells, and ultrastructural study revealed Birbeck granules in them. Vinblastine therapy reduced jaundice and high fever, and diabetes insipidus was controlled. Clinical, immunohistological, and cytological features suggested Letter-Siwe disease with jaundice, which responded well to treatment.