Mustafa Buğra COŞKUNER, Tevfik ÖZLÜ, Yılmaz BÜLBÜL

Nadir bir hastalık; spontan pnömotoraks ile başvuran Erdheim-Chester hastalığı

Erdheim-Chester hastalığı (ECD) sebebi bilinmeyen köpüksü histiyositlerinorgan tutulumu ile karakterize non-Langerhans hücreli histiyositozis formudur. ECD tanısı doku biyopsilerinde tipik olarak CD68+ CD1a- histiyositlerintespitini içerir. Bu karakteristik histiyositler ECD olgularında hemen her dokuda bulunabilir. Spontan pnömotoraks ve nörolojik tutulum ile başvuran ilginçbir ECD olgusunu sunuyoruz.

A rare disease; Erdheim-Chester disease presenting with spontaneous pneumothorax

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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