Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis is a rare condition that can affect any organ of the body. Patients of all ages may present to the dermatologist and it is important to make the diagnosis as quickly as possible, because time from presentation to diagnosis is of prognostic importance in adults with the disease. Langerhans cell histiocytosis is now classified as a class I histiocytosis and research into this disease has been very active over the past 10 years. We now know that the disease represents a clonal expansion of a Langerhans cell, which shows phenotypic evidence of activation. The tissue damage induced by the disease appears to be related to local cytokine release. In single system disease, Langerhans cell histiocytosis is responsive to local therapy but, in resistant single system disease or in multisystem disease, etoposide is the most effective monochemotherapy. Some patients will need maintenance treatment with azathioprine or 6-mercaptopurine with or without methotrexate. In such cases, physicians who are used to treating chronic and relapsing diseases and who have experience with these drugs, such as dermatologists, are the most appropriate to manage patients with Langerhans cell histiocytosis.