A Rare Cause of Abdominal Pain: Erdheim-Chester Disease

Arjun Chatterjee; Jaime de la Fuente; Karen L Rech; Naoki Takahashi; Shounak Majumder

doi:10.14309/crj.0000000000001049

A Rare Cause of Abdominal Pain: Erdheim-Chester Disease

ACG Case Rep J. 2023 Jun 8;10(6):e01049. doi: 10.14309/crj.0000000000001049. eCollection 2023 Jun.

Authors

Arjun Chatterjee¹, Jaime de la Fuente², Karen L Rech³, Naoki Takahashi⁴, Shounak Majumder²

Affiliations

¹ Department of Internal Medicine, Cleveland Clinic, Cleveland, OH.
² Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN.
³ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
⁴ Department of Radiology, Mayo Clinic, Rochester, MN.

Abstract

A 65-year-old man presented with hematuria, night sweats, nausea, intermittent nonbloody diarrhea, and abdominal pain. Computed tomography angiogram with enterography showed retroperitoneal fibrosis surrounding both kidneys and ureters without any evidence of vascular obstruction or hydronephrosis. Laparoscopic biopsy demonstrated fibroadipose tissue involved by a subtle histiocytic infiltrate in a background of marked fibrosis, scattered lymphocytes, and plasma cells. The histiocytes strongly expressed CD163, Factor XIIIa, and BRAF V600E. He was diagnosed with Erdheim-Chester disease, a rare histiocytic neoplasm uncommonly presenting with gastroenterological manifestations.

Keywords: Erdheim-Chester disease; IgG4 disease; IgG4 pancreatitis; histiocytic neoplasms; retroperitoneal mass.

Publication types

Case Reports