DOI: 10.36347/sjmcr.2020.v08i03.026

Pages: 352-355

Erdheim-Chester disease is a rare systemic Langrehansian histiocytosis. It is diagnosed on the basis of a bundle of clinical, radiological and histological arguments. Its confirmation is based on a precise immunological profile. The bone involvement is almost constant, characterized by osteocondensing lesions more often at the metaphysso-diaphyseal level of the long bones of the lower limbs. We report the case of a 37-year-old female patient with chronic left jugular swelling. The CT scan revealed a mandibular osteolytic lesion including the cortex without extension towards the soft parts. The diagnosis of EDC was established based on the histological aspect of the lesion and confirmed by the immunohistochemical study.