A Fatal Case of Erdheim-Chester Disease with Hepatic Involvement. ACG Case Rep J 2017;4:e95
Date
08/12/2017Pubmed ID
28798943Pubmed Central ID
PMC5541758DOI
10.14309/crj.2017.95Abstract
Erdheim-Chester disease (ECD) is a rare form of systemic histiocytosis, typically presenting with striking osseous involvement characterized by bilateral osteosclerosis and involvement of organs such as the lung, pituitary gland, heart, and brain. Liver involvement with ECD is extremely uncommon. We report a 56-year-old woman presenting with newly diagnosed cirrhosis and signs concerning for intra-abdominal malignancy, including omental caking and peritoneal thickening. Liver biopsy demonstrated xanthogranulomatous infiltration from ECD. The patient showed initial improvement with interferon therapy, but she developed severe depression, which led to the discontinuation of the treatment. Shortly afterward, she died from progressive liver dysfunction resulting in hepatorenal syndrome.
Author List
Balasubramanian G, Modiri A, Affi M, Hagen CE, Batdorf B, Oshima K, Michaelis L, Saeian KAuthors
Gokulakrishnan Balasubramanian MD Associate Professor in the Medicine department at Medical College of WisconsinLaura Michaelis MD Chief, Professor in the Medicine department at Medical College of Wisconsin
Kia Saeian MD Professor in the Medicine department at Medical College of Wisconsin