Reversible hepatomegaly and diabetes mellitus in an adult with disseminated histiocytosis X

Histiocytosis X rarely disseminates in an adult. The authors describe an unusual patients who presented with multiple areas of cutaneous and bone involvement. During the course of his disease he developed massive hepatomegaly. Aggregates of vacuolated histiocytes were found on liver biopsy. He subsequently developed diabetes mellitus complicated by ketoacidosis. Both his hepatomegaly and diabetes resolved spontaneously. No pancreatic nor pituitary abnormalities were identified. The combination of histiocytosis X, hepatomegaly, and diabetes mellitus has not been previously reported. The medical literature is reviewed with an emphasis on disseminated histiocytosis X in adults and the mechanism of glucose intolerance in liver disease.