Erdheim Chester disease


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  • Clinical

    • mean age 52
    • site: bone
    • other sites: sinus, large vessels, retroperitoneum, heart/lung, CNS/pituitary, skin, orbit

  • Histology

    • foamy histiocytes
    • touton giant cells

  • Stains

    • pos: CD68, CD163, CD14, factor XIIIa, fascin
    • neg: S100, CD1a, langerin
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