Abstract

Langerhans cell histiocytosis (LCH) is the latest terminology for a disorder of reticulo-endothelial system, previously known as histiocytosis X, and marked by aberrant proliferation of bone marrow derived Langerhans cells with variable inflammatory infiltrate including neutrophils, lymphocytes, plasma cells, eosinophils, and multinucleated giant cells. Although rare, the disorder frequently inflicts children with peak incidence recorded in 2-4 years age group. LCH is rare in adults. A22-year adult male presented with the complaint of teeth mobility. Orthopantomogram (OPG) revealed multiple extensive osteolytic lesions destroying both jaws. Giant cell granuloma, Paget's disease and LCH, along with other vanishing bone disorders (Gorham Stout disease and Winchester syndrome) were suspected differentials. Multiple incisional biopsies were performed and a diagnosis of LCH was confirmed by routine histopathological analysis followed by immunohistochemical staining for S-100 and CD1a.

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