Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis is a rare disease in children, its initial presentation is variable, the clinical course, prognosis and survival are mostly unpredictable. LCH is a proliferative disorder where pathologic Langerhans cells accumulate in a variety of organs. Historically, the nomenclature regarding this entity has been confusing because the disease was subcategorized as eosinophilic granuloma, Hand-Schüller-Christian disease and Abt-LettererSiwe disease, simply based upon the different clinical manifestations. This nomenclature remains valuable and preserves the important historic perspective of these disorders. Currently, LCH can be divided according to disease extent and localisation of the disease at the time of evaluation. The following clinical categories have been defined: single- or multi-system disease. This article summarizes the classification, history, pathophysiology, diagnostic criteria, different clinical manifestations, treatment, prognosis and long-term sequelae of LCH in children.