Three patients with biopsy proven histiocytosis X who developed a clinical and pathological picture compatible with sclerosing cholangitis are reported. In one patient, operative biopsy of the common bile duct revealed histiocytosis X in the granulomatous/xanthomatous phase. At necrospy, however, only fibrosis of the biliary tree was seen, a picture consistent with sclerosing cholangitis. Fibrotic obstruction of the biliary tree led to death from liver failure in all three patients. The aetiology of primary sclerosing cholangitis is unknown and may be multifactorial. Perhaps involvement of the biliary tree by histiocytosis X is one cause.
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