Erdheim-Chester disease

Discussion:

Erdheim-Chester disease is non-Langerhans cell histiocytosis resulting in multiple organs xanthomatous infiltration, usually presents with bone pain, diabetes insipidus, exophthalmos, interstitial lung disease, and renal impairment.

The differential diagnosis of this pattern of renal involvement includes retroperitoneal fibrosislymphoma and extramedullary hematopoiesis.

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