• Stefan Krüger, Cornelia Krop, Thomas Wibmer, Sandra Pauls, Felix M Mottaghy, Christian Schumann, and Vinzenz Hombach.
• Innere Medizin II, Universitätsklinikum Ulm, Ulm. s.krueger@uniklinik-ulm.de
• Med Klin. 2006 Jul 15; 101 (7): 573-6.

BackgroundErdheim-Chester disease is a rare non-Langerhans' cell histiocytosis of unknown etiology with typical osteosclerotic long-bone findings. However, clinical symptoms are mostly due to disease involvement of other tissues, particularly of the lung, heart and retroperitoneum.Case ReportA 58-year-old man presented with fatigue, diffuse pain of the lower extremities, dyspnea, and a dry cough. Physical examination was unremarkable. Chest X-ray revealed an extensive bilateral pulmonary inter interstitial process. Computed tomography demonstrated pulmonary fibrosis, periaortic fibrosis of the thoracic aorta, and retroperitoneal fibrosis. The diagnosis of Erdheim-Chester disease was confirmed by minimally invasive lung biopsy. Steroid therapy was not tolerated. Following a stable interval of 18 months there was a disease progression, which could be stabilized after the initiation of cyclophosphamide therapy.ConclusionIn patients with extensive pulmonary fibrosis and coincidence of other organ manifestations such as periaortic or retroperitoneal fibrosis and particularly in case of symmetrical osteosclerotic bone lesions, Erdheim-Chester disease should be considered. Immunosuppressive therapy can lead to a stabilization or even improvement of the disease.

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