Erdheim-Chester disease and knee pain in a dialysis patient

Erdheim-Chester disease is a rare inflammatory condition characterized by a non-Langerhans histiocytic infiltration, involving the skeleton, nervous system, viscera, retroperitoneum and elsewhere. The aetiology is unknown. Positron emission tomography shows areas of involvement. We managed a dialysis patient with knee pain; a bone marrow specimen showed typical CD68 positive, but CD1a negative cells. We initiated interferon-α therapy although other options remain open. In our patient, the simultaneous presence of secondary hyperparathyroidism with tumorous calcifications provided an interesting additional differential diagnostic possibility regarding skeletal pain.