Letterer-Siwe disease is one of Langerhans cell histiocytosis, with Hand-Schuller-Christian disease and eosinophilic granuloma, characterized by proliferation of Langevha is cell. The clinical course of Letterer-Siwe disease is acute fulmunant of casionally fatal, involving skin and many other internal organs, such as the lymph node, liver, spleen, ung and bone. We present 3 cases of Letterer-Siwe disease with characteristic cutaneous findings and revealed Langerhans granules by imrriunohistochemical stain and electronmicrc scopic examination. All patients died in spite of combined chernotherapy.
Eosinophilic Granuloma ; Histiocytosis, Langerhans-Cell ; Humans ; Liver ; Lymph Nodes ; Skin ; Spleen