Abstract
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–Schuller–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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