A Case of Letterer-Siwe Disease in Adult / 대한피부과학회지

ABSTRACT

Letterer-Siwe disease is a one of Langerhans cell histiocytosis and characterized by proliferation of Langerhans cells. It's clinical features are onset of infancy, hemorrhagic crusted papules and petechiae, hepatomegaly, lymphadenopathy, localized bone defect, and fatal outcome. We report a 38-year-old woman with hepatomegaly, lymphadenopathy, scaly papules and petechiae on the trunk. The eletronmicroscopy showed a tennis racquet shaped Birbeck granules in cytoplasm of histiocyte. Systemic treatment with etoposide, cyclophosphamide, prednisone, and vincristine was effective.