Oral radiographic and clinico-pathologic presentation of Erdheim-Chester disease: a case report.

A 69-year-old woman with unexplained fever and weakness was referred for evaluation of a solitary mandibular swelling adjacent to a severely resorbed edentulous mandibular ridge. The patient had coexisting craniofacial-skeletal lesions and diabetes insipidus. Histological and immunohistochemical staining of sections from mandibular lesions confirmed the rare diagnosis of Erdheim-Chester disease. The absence of cardiac, pulmonary, renal, and major neurological manifestations was suggestive of a diagnosis at an early stage of the disease. Early diagnosis has been rare with less than 100 reported cases. A review of the literature revealed only 2 cases that report detailed maxillomandibular radiographic findings. A seemingly benign clinical presentation of a potentially grave disease that presents with an osteolytic-sclerotic oral radiographic picture is reported.