Abstract:Langerhanshistiocytosis is a rare disorder with a heterogeneous clinical presentation. It is characterized by an abnormal proliferation of Langerhans cells in different tissues.The clinical manifestations depend on the number of lesionsand their extension to the soft tissues.The organs affected by the disease, besides the head and the neck, may be the skin, long bones and the digestive system. Imaging confirms osteolytic lesions. The definitive diagnosis is made by biopsy with histological study.
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