Unifocal Langerhans Cell Histiocytosis

Langerhans cell histiocytosis is a rare disease which is more common in the childhood. Clinical presentation may vary ranging from single to multiple organ involvement. Skeleton is often involved and in the maxillofacial region, it affects the mandible more than the maxilla. Radiographically, it may present as a well-defined radiolucency or an ill-defined radiolucency. The presence of Langerhans cells in an inflammatory background with prominent eosinophils gives a clue to the diagnosis. Confirmation is by positive immunohistochemical staining with S100, CD1a and CD207. Here, we report a case of a 7-year-old female patient who presented with a swelling in the left mandibular region which was diagnosed as unifocal Langerhans cell histiocytosis after complete evaluation.