Erdheim-Chester disease. An update

Abstract

Erdheim-Chester disease is a rare non-Langerhans cell histiocytic disorder characterized by abnormal multiplication of histiocytes or tissue macrophages. It is most commonly characterized by multifocal osteosclerotic lesions of the long bones demonstrating sheets of foamy histiocytes, with or without histiocytic infiltration of extraskeletal tissues such as the skin, pituitary gland, retroperitoneum, kidneys and heart. Most cases are diagnosed in adulthood, between ages 40 and 60 years (mean, 53 years), with a 3-fold frequency in males than females. Sporadic cases in children have been reported. This review discusses the clinicopathological features, diagnosis and current treatment of this rare, non-Langerhans cell histiocytosis.