Langerhans cell histiocytosis(LCH) is a disease characterized by clonal proliferation and excessive accumulation of disseminated forms of dendritic Langerhans cells. The aims of thestudy were to describe clinico-pathological presentations of thirteen new cases of LCH of the oral cavity and to present its differential diagnoses. The majority of 61.53% (8/13) of cases occurred in infants and adolescents with female predominance (61.53%). Out of all the lesions,61.53 % occurred intraosseously;with majority 53.82% (7/13) presenting on the posterior mandible and 15.38% (2/13)in the anterior mandible.Five lesions extended to involve more than one site. Radiologically all intraosseous lesions presented as osteolytic lesions with ill-defined or well defined margins. A wide range of clinical differential diagnoses including malignancies have been proposed indicating the aggressive clinical presentations exhibited by some lesions. Histopathologically, all lesions at first glance resembled an infection/chronic inflammatory process and contained mixed inflammatory cells including eosinophils. Deep fungal infections and traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) were the two main histopathological differential diagnoses that were considered for hard tissue and soft tissue lesions respectively. Round mononuclear cells, containing eosinophilic cytoplasms and peripheral indented cerebriform nuclei were only observed under higher magnification. Immunohistochemical investigations with S-100 antibody reveled nuclear and cytoplasmic positivity in mononuclear cells confirming these cells as Langerhans cells. All the patients were treated with complete surgical excision with or without chemo/radiotherapyand are under follow up care. In conclusion, as LCH of oral cavity may have a broad spectrum of clinico-pathological presentations, high degree of clinical awareness is required to arrive at the definitive diagnosis. The histopathological diagnosis is reliable with immunohistochemical confirmation of the Langerhans cell. However, awareness of its differential diagnosis is important to avoid misdiagnosis; resulting in incorrect management.
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