Korean J Dermatol.  1994 Feb;32(1):186-192.

Three Cases of Letterer - Siwe Disease

Abstract

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.

Keyword

Langerhans cell histiocytosis; Langerhans granules; Letterer-Siwe disease

MeSH Terms

Eosinophilic Granuloma
Histiocytosis, Langerhans-Cell
Humans
Liver
Lymph Nodes
Skin
Spleen
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