Erdheim-Chester disease. Case report and review of the literature.
Erdheim-Chester disease is a distinctive pathologic and radiographic entity characterized by bilateral, symmetric sclerosis of the metaphyseal regions of long bones and infiltration of foamy, lipid-laden histiocytes. Clinically, it ranges from an asymptomatic, focal process to a fatal, systemic disease. All previously reported cases of Erdheim-Chester disease are reviewed and a new case with diabetes insipidus, partial hypopituitarism, histiocytic skin lesions, and retro-orbital tumors is presented. An 11-year follow-up reveals progression from a benign, limited process to a progressive, systemic disease. Patients with Erdheim-Chester disease show markedly disparate clinical courses and some features similar to Hand-Schüller-Christian disease. They need careful monitoring and further attempts at treatment.
D000328:Adult; D001847:Bone Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003919:Diabetes Insipidus / Q000175:diagnosis; D005500:Follow-Up Studies; D006801:Humans; D007018:Hypopituitarism / Q000150:complications; D008206:Lymphatic Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008297:Male; D010014:Osteolysis / Q000150:complications