Letterer-Siwe disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Letterer-Siwe disease is a condition where histiocytes proliferate in the body. It is sometimes classified as a form of Langerhans cell histiocytosis, or as a disseminated form of histiocytosis X. It is most commonly seen in children less than two years old.

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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