Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases

Abstract

Langerhans cell histiocytosis (LCH) is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. The head and neck are affected in almost 90% of cases. Diagnosis is made by means of histopathological analysis, and imaging studies are necessary in order to determine extent of the disease. There are no controlled trials proposing an optimal treatment protocol for LCH. Prognosis in adults is generally good due to the slow evolution of the disease and its favourable response to treatment. In our report, we present three cases of LCH in patients aged 16, 24, and 28 years respectively, with primary manifestation in the maxillofacial area. A literature review was also conducted.