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Langerhans cell histiocytosis is a rare disorder of unknown etiology characterized by an aggregation of proliferating histiocytelike and other inflammatory cells.1 Some evidence suggests that it may represent a clonal disorder, supporting a neoplastic origin rather than a polyclonal reactive disease.2,3 The Langerhans cell is a dendritic cell that is derived from the bone marrow.4 Langerhans cell histiocytosis may present locally (previously known as eosinophilic granuloma of bone) or as a multifocal disease that affects only the skeletal system or other tissues and organs, such as the lungs, liver, and skin (previously known as Hand-Schuller-Christian and Letterer-Siwe diseases). The temporal bones can be affected by LCH as a solitary lesion or as part of a multisystemic involvement in 15% to 61% of patients.5,6
Diagnosis Quiz Case 1. Arch Otolaryngol Head Neck Surg. 2001;127(1):80. doi:
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