Multifocal eosinophilic granuloma involving the common bile duct: histologic and cholangiographic findings

The multifocal eosinophilic granuloma syndrome (Hand-Schüller-Christian disease) is characterized histologically by focal accumulations of histiocytes and eosinophils. The clinical manifestations of this syndrome reflect the site and extent of these focal accumulations which are usually found in membranous bone, skin, and lung. Significant hepatobiliary disease due to this disorder has not been previously described. We are reporting an adult female with the multifocal eosinophilic granuloma syndrome who developed a mixed histiocytic and eosinophilic infiltration of the common bile duct which resulted in obstructive jaundice. Therapeutic attempts have failed to reverse this process, and the patient has shown progressive intrahepatic biliary tract destruction.