ABSTRACT

Langerhans cell histiocytosis (LCH), also formerly known as histiocytosis X and currently regarded as a myeloid neoplasm, is a disease characterized by proliferation of cells exhibiting phenotypic characteristics of Langerhans cells. LCH includes a wide range of clinical presentations from a single system involvement such as the skin or bone to a multifocal disease involving liver, lungs, bone marrow and CNS. Although the pathogenesis of LCH remains enigmatic, the disease seems to be associated with abnormalities in the biology of Langerhans cells and macrophage infiltrates in the lesions.

Here we present a case of LCH of a 4– year– old male child who presented with a swelling of the body of mandible on the left side.