Dinkar Ajit D, Spadigam Anita, Sahai Sharad
Oral Medicine, Diagnosis, and Radiology Department, Goa Dental College and Hospital (Government of Goa), Bambolim, Goa, India.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007 May;103(5):e79-85. doi: 10.1016/j.tripleo.2006.11.006. Epub 2007 Feb 20.
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.
一名69岁女性，伴有不明原因发热和虚弱，因下颌严重吸收的无牙牙槽嵴旁出现孤立性下颌肿胀而前来接受评估。该患者同时存在颅面骨骼病变和尿崩症。下颌病变切片的组织学和免疫组化染色确诊为罕见的厄尔德海姆-切斯特病。无心脏、肺部、肾脏及主要神经方面的表现提示疾病处于早期阶段。早期诊断极为罕见，报告病例不足100例。文献回顾仅发现2例报告了详细的颌面部影像学检查结果。本文报告了一种潜在严重疾病看似良性的临床表现，其口腔影像学表现为溶骨性-硬化性改变。
