title: Oral radiographic and clinico-pathologic presentation of Erdheim-Chester disease: a case report.
abstract: 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.
authors.affiliation: Oral Medicine, Diagnosis, and Radiology Department, Goa Dental College and Hospital (Government of Goa), Bambolim, Goa, India.
authors.forename: Ajit D
authors.initials: AD
authors.lastname: Dinkar
authors.forename: Anita
authors.initials: A
authors.lastname: Spadigam
authors.forename: Sharad
authors.initials: S
authors.lastname: Sahai
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006801:Humans; D008336:Mandibular Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D010026:Osteosclerosis / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D012886:Skull / Q000000981:diagnostic imaging / Q000473:pathology