랑게르한스 세포 조직구증에 속발한 Erdheim-Chester 병 -1예 보고-

초록

Erdheim-Chester disease(ECD) is a rare systemic disorder of unknown etiology, likely neoplastic, characterised by xanthogranulomatous infiltrates of foamy, lipid-laden histiocytes in all involved sites, principally bone, retroperitoneum, lung, pleura, pericardium, retroorbital tissue and brain including skin. This disease belongs to class II(non-Langerhans cell) histiocytosis and differs from Langerhans cell histiocytosis(LCH) for clinical, histologic and immunohistochemical features. We report a patient with LCH who was subsequently shown to have ECD.