Related Experiment Video
Updated: Jan 4, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[A rare disease; Erdheim-Chester disease presenting with spontaneous pneumothorax]
Mustafa Buğra Coşkuner1, Tevfik Özlü1, Yılmaz Bülbül1
1Department of Chest Diseases, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.
Abstract:
Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, it is characterized by organ infiltration of foamy histiocytes. Diagnosis of ECD involves the analysis of histiocytes in tissue biopsies: these are typically CD68+ CD1a-. These characteristic histiocytes may be found in almost any tissue in cases of ECD. We present an interesting case of ECD that was presented by spontaneous pneumothorax and neurological involvement.
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