Author + information
- Received November 8, 2013
- Accepted November 19, 2013
- Published online March 25, 2014.
- Masa-aki Kawashiri, MD, PhD∗,
- Hatsue Ishibashi-Ueda, MD, PhD†,
- Akira Funada, MD, PhD‡,
- Tsuyoshi Yoshimuta, MD, PhD∗,
- Tetsuo Konno, MD, PhD∗,
- Kenshi Hayashi, MD, PhD∗ and
- Masakazu Yamagishi, MD, PhD∗
A 66-year-old Japanese woman presented with a 3-year history of pericardial effusion. In addition, she had hemiplegia 1 month prior. On physical examination, xanthoma was observed in both eyelids (A, arrow). Brain magnetic resonance imaging demonstrated multiple subdural tumors (B, arrow). Interestingly, irregularly thickened pericardium with effusion was observed by means of cardiac magnetic resonance imaging, which also demonstrated fibrosis around the right atrium (C, arrow). Histology of the biopsied pericardium showed the presence of inflammatory cells including giant cells (D) and macrophages with CD68+ (D, inset) and CD1a–, suggesting non-Langerhans cell histiocytosis such as Erdheim-Chester disease. Erdheim-Chester disease is a rare disease characterized by the abnormal multiplication of histiocytes, presenting orthopedic, neurological, and cardiovascular symptoms that usually do not respond to treatment. The patient died of heart failure 6 months later. The appearance of xanthoma is suggestive for the presence of histiocytosis when other lipid disorders can be excluded.
- Received November 8, 2013.
- Accepted November 19, 2013.
- American College of Cardiology Foundation