Erdheim Chester Histiocytosis: About a New Case Reveled after Ten Years History of Diabetes Insipidus

Erdheim-Chester Disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by the proliferation of lipid-rich histiocytes and foam-like tissue cells. Here, we present a case report of a 31-year-old male patient with ECD who initially presented with nonspecific symptoms including diabetes insipidus. Histopathological analysis confirmed the diagnosis, showing typical histological features of ECD. Treatment with Anakinra and corticosteroids led to significant improvement in symptoms. Additionally, we provide a comprehensive literature review summarizing the clinical manifestations, diagnostic criteria, pathological features, and treatment options for ECD. This case highlights the importance of considering ECD in the differential diagnosis of patients presenting with multisystem involvement and underscores the challenges in managing this rare disease.