Letterer-Siwe disease

Letterer-Siwe disease

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

It is named for Erich Letterer and Sture Siwe.

Presentation

Symptoms include lymphadenopathy, hepatosplenomegaly, and seborrhea-like lesions on the skin.

Prognosis

Untreated, the disease is fatal. The five-year survival rate with treatment is fifty percent.

References

External links

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