Letterer-Siwe disease is one of Langerhans cell histiocytosis, with Hand-Schuller-Christian disease and eosinophilic granuloma, characterized by proliferation of Langerhans cell.
The clinical course of Letterer-Siwe disease is acute fulmunant, occasionally fatal, involving skin and many other internal organs, such as the lymph node. Liver, spleen, lung and bone.
We present 3 cases of Letterer-Siwe disease with characteristic cutaneous findings and revealed Langerhans granules by immunohistochemical stain and electronmicroscopic examination. All patients died in spite of combined chemotherapy. (Kor J
Dermatol
1994 ; 32(1) : 186~192)
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