Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis characterized by xanthogranulomatous infiltration of foamy histiocytes surrounded by fibrosis. ECD may be asymptomatic or present as a multi-systemic disease with life-threatening manifestations, most commonly involving the skeletal system. Immunohistochemical staining demonstrates cells that are CD68+, CD1a-, and S100- with an absence of Birbeck granules. We report a case of a 69-year old male patient who presented with neurological symptoms - eventually thought to be separate to his diagnosis of ECD. It represents the ability to diagnose ECD based just on radiological findings in an otherwise asymptomatic individual.
Keywords: Coated aorta sign; Erdheim-Chester Disease; Hairy kidney sign; Histiocytosis.
© 2020 Published by Scientific Scholar on behalf of Journal of Clinical Imaging Science.