Letterer-Siwe disease
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| ICD-10 | C96.0 |
|---|---|
| ICD-9 | 202.5 |
| OMIM | 246400 |
| DiseasesDB | 5906 |
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 diseminated 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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