Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis characterized by xanthogranulomatous infiltrates of foamy CD68 positive, CD1a and S-100 protein negative histiocytes, surrounded by fibrosis. This disease can affect multiple organ s...
Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis characterized by xanthogranulomatous infiltrates of foamy CD68 positive, CD1a and S-100 protein negative histiocytes, surrounded by fibrosis. This disease can affect multiple organ systems, as many cases described lesions of long bones, lung, heart, and CNS involvement. However, colon involvement in ECD is exceedingly rare. Only one case of gastrointestinal involvement in ECD, discussing the endoscopic findings, has been reported to date. Herein, we present a case of ECD with predominant colon involvement, along with unique endoscopic images and consequent pathologic confirmation, as well as a current review of this disease.