Erdheim-Chester disease with long-standing diabetes insipidus and generalized edema

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis. ECD is detected more frequently due to increased awareness of healthcare providers and improved diagnostic tools. This report describes a 51-year-old woman with a history of weakness, bone pain, xanthelasma palpebrarum, and diabetes insipidus. ECD is a multisystemic condition with a poor prognosis. This disease should be considered in patients with diabetes insipidus, bone pain, and multiorgan involvements.