Oral Findings of Erdheim-Chester Disease: A Case Report and Review of the Literature

Erdheim-Chester Disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by histiocytic infiltration of multiple organ systems, including the bony skeleton, central nervous system, cardiovascular system, lungs, and kidneys. Manifestations of the disease are highly variable, ranging from focal disease to multisystem involvement. Oral manifestations are exceedingly rare, with only 10 known reports of ECD involving the jaws. The objective of this case report is to highlight the radiographic, clinical, and histological features of ECD involving the maxillofacial complex. A 35-year-old male admitted for sepsis secondary to pyelonephritis and suspected diabetes insipidus received an extensive workup for ECD versus IgG4 disease versus lymphoma. The oral-maxillofacial surgery service was consulted for biopsy of maxillary lesions noted to have increased activity on positron emission tomography. Intraoral biopsy yielded the characteristic histopathological features of ECD, confirming the diagnosis. We compare the clinical, radiographic, and microscopic features of ECD with past findings, contributing to the profile of this rare disease. (c) 2021 American Association of Oral and Maxillofacial Surgeons