Langerhans cells (LC) are a group of antigen-presenting, migratory and non-proliferative cells belonging to a group of leukocytes called dendritic cells (DC). In normal scenarios, LC is found in the suprabasal layer of the epidermis and in bronchial, urogenital, and oropharyngeal mucosas. 1 Langerhans cell histiocytosis (LCH) is a condition characterized by abnormal proliferation and infiltration of marrow-derived LC that may be diagnosed in oral and maxillofacial tissues. 2,3 Therefore, the aim of the present report was to describe the main characteristics of this uncommon disease, with emphasis on its clinical manifestations, microscopic features, and the therapeutic approaches currently available. This study forms part of a special issue published by the Journal of Oral Pathology and Medicine covering the most important aspects of hematolymphoid lesions and neoplasms affecting the oral cavity and neighboring structures.
| OVERVIE WThe first case of LCH was described by Hand in 1893. 4 More than hundred years later, LCH still challenges physicians from the diagnosis to treatment due to reasons such as the rare occurrence of this condition and its broad clinical manifestations. 5 Following the first description, LCH was called as monocyte-macrophage system disease because of the identification of histiocyte-like cells in affected tissues. In this respect, Hand-Schuller-Christian syndrome, Letterer-Siwe disease,