Langerhans Cell Histiocytosis
Langerhans cell histiocytosis (LCH), previously referred to as histiocytosis X, is a rare clonal disorder of Langerhans cell proliferation, involving the skin, bone, and other organs. The disease family consists of the syndromes originally described as eosinophilic granuloma, Hand–Schüller–Christian disease, and Letterer–Siwe disease, a fatal and disseminated form of Langerhans cell histiocytosis which is most commonly seen in children less than two years old. Modern classification of LCH consists of single system versus multisystem and unifocal versus multifocal.
Most patients diagnosed with LCH are children with a peak percentage of diagnoses occurring between 1 and 3 years of age. The incidence of LCH has been estimated to be five cases per million per year in children. It appears to be more common in boys than in girls (1.2–2:1). The incidence of LCH in adults is thought to be one-half of that in children....
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- Donadieu J, Egeler RM, Pritchard J (2005) Langerhans cell histiocytosis: a clinical update. In: Weitzman S, Egeler RM (eds) Histiocytic disorders of children and adults; basic science, clinical features and therapy. Cambridge University Press, Cambridge, pp 95–129Google Scholar