Biology of Langerhans cells and Langerhans cell histiocytosis

Langerhans cells (LC) are epidermal dendritic cells (DC). They play an important role in the initiation of immune responses through antigen uptake, processing, and presentation to T cells. Langerhans cell histiocytosis (LCH) is a rare disease in which accumulation of cells with LC characteristics (LCH cells) occur. LCH lesions are further characterized by the presence of other cell types, such as T cells, multinucleated giant cells (MGC), macrophages (MPhi), eosinophils, stromal cells, and natural killer cells (NK cells). Much has been learned about the pathophysiology of LCH by studying properties of these different cells and their interaction with each other through cytokines/chemokines. In this review we discuss the properties and interactions of the different cells involved in LCH pathophysiology with the hope of better understanding this enigmatic disorder.