Coşkuner Mustafa Buğra, Özlü Tevfik, Bülbül Yılmaz
Department of Chest Diseases, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.
Tuberk Toraks. 2019 Sep;67(3):225-230. doi: 10.5578/tt.68541.
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.
Erdheim-Chester病（ECD）是一种病因不明的罕见非朗格汉斯组织细胞增多症，其特征是泡沫状组织细胞浸润器官。ECD的诊断涉及对组织活检中的组织细胞进行分析：这些细胞通常CD68阳性、CD1a阴性。在ECD病例中，几乎任何组织都可能发现这些特征性组织细胞。我们报告一例以自发性气胸和神经受累为表现的有趣ECD病例。
