Abstract
A 20-year-old woman was admitted due to anorexia, general malaise and fever. She was treated with vasopressin for diabetes insipidus at the age of eighteen. On admission, physical examination revealed generalized icterus and hepatomegaly. Laboratory data showed anemia and increased hepatobiliary enzymes. At laparoscopy, diffuse granulomatous lesions of the liver were noted. Histologically, infiltration of the histiocytic cells was found at the portal area. Immunohistochemically, these cells were negative for lysozyme stain, but were positive for S100 protein stain. An electron microscopic examination revealed the presence of Birbeck granule in histiocytic cells. On the basis of these findings, Histiocytosis X was diagnosed. After administration of vinblastine, diabetes insipidus, jaundice and liver function were improved.
