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Letterer-Siwe disease is a type of histiocytosis[1] (a condition where histiocytes proliferate in the body.) It is sometimes classified as a form of Langerhans cell histiocytosis,[2] or as a form of histiocytosis X.[3] It is most commonly seen in children less than two years old.
It is named for Erich Letterer and Sture Siwe.[4][5][6]
Symptoms include lymphadenopathy, hepatosplenomegaly, and seborrhea-like lesions on the skin.
Untreated, the disease is fatal. The five-year survival rate with treatment is fifty percent.
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