Kalp ve beyin tutulumlu Erdheim-Chester hastalığı

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of systemic histiocytosis of unknown etiology that progresses with multiple organ involvement. It is characterized by a xanthomatous infiltration showing positive staining with CD68 and negative staining with CD1a in tissues. We report a 62 year-old case complaining of malodor, in the brain magnetic resonance imaging of whom a mass was found at the clivus and leptomeningeal involvement was determined. The accompanying intracardiac mass was excised and the case was diagnosed histopathologically as ECD. We present the unusual clinical symptoms along with histopathologic and radiodiagnostic findings and discuss the differential diagnosis of this rare disease.Keywords: Erdheim-Chester disease, magnetic resonance imaging, cardiac involvement, neurological signs. Öz Erdheim-Chester hastalığı (ECD) etyolojisi bilinmeyen, multipl organ tutulumu ile seyreden