Erdheim-Chester disease and concomitant tuberculosis successfully treated with chemotherapy and long-term steroids.

Erdheim-Chester disease (ECD) is a rare histiocytosis usually affecting the skeletal system, but visceral organs and central nervous system involvement are common as well. Probability exists that immunomodulatory therapies and disorders can play a role in clinical course of the disease. Because of rarity of the disorder, it is hard to classify it and standardize the treatment options, but, according to published material and our experience, cytotoxic chemotherapy and long-term steroids have therapeutic benefit. Although this approach can probably be accepted as standard of care management, novel therapeutic modalities should be explored, and pathogenesis and disorder classification should be cleared out as well. The case of ECD affecting skeletal system and lungs and concomitant laryngeal tuberculosis successfully treated with chemotherapy and long-term steroid therapy is presented.