A Case of Letterer-Siwe Disease in Adult.
- Author:
Sung Joo LEE
1
;
Duck Hwan WON
;
Gwang Seong CHOI
;
Sang Wahn KOO
;
Joo Heung LEE
;
Young Keun KIM
Author Information
1. Department of Dermatology, Inha University College of Medicine, Inchon, Korea.
- Publication Type:Case Report
- Keywords:
Letterer-Siwe disease;
Langerhans cells;
Adult
- MeSH:
Adult*;
Cyclophosphamide;
Cytoplasm;
Etoposide;
Fatal Outcome;
Female;
Hepatomegaly;
Histiocytes;
Histiocytosis, Langerhans-Cell*;
Humans;
Langerhans Cells;
Lymphatic Diseases;
Prednisone;
Purpura;
Tennis;
Vincristine
- From:Korean Journal of Dermatology
2000;38(12):1688-1690
- CountryRepublic of Korea
- Language:Korean
-
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.
