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CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 4  |  Page : 416-419

Hand–Schuller–Christian disease with condylar aplasia: A rare and unique case


Department of Oral medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Hemavathy Bhaskar Yesupogu
Department of Oral medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.JIAOMR_155_15

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Langerhans cell histiocytosis (LCH) is characterized histologically by a monoclonal proliferation of large mononuclear cells accompanied by a prominent eosinophil infiltrate. The pathogenesis of LCH is unknown. Hand–Schüller–Christian (HSC) disease is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in various organs. The clinical manifestations arise as a result of accumulation and infiltration of the Langerhans cells in organs and tissues. HSC is characterized by a classic triad of exophthalmos, diabetes insipidus, and lytic lesions in the skull. It is usually manifested in children but can also affect adults. Here, we present a case of HSC disease in a 12-year-old boy with oral manifestations.


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