랑게르한스세포 조직구종에 속발한 Erdheim-Chester 병 -1예 보고-

초록

Erdheim-Chester disease (ECD) is a rare systemic disorder of unknown etiology, likely neoplastic, characterised by xantogranulomatous 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. The patient was a 42-year-old woman complained of a severe dyspnea and or thopnea associated with features of interstial infiltration of lung with bilateral pleural effusion and diabetus insipidus. Five years previously, she was diagnosed as pulmonary tuberculosis and LCH accomanied by tuberculous lymphadenopathy of neck nodes. She underwent consecutive punch biopsy of the papule from the abdominal skin that was also diagnosed as LCH. There was no response to 12 cycles of chemotherapy. On current admission, punch biopsy from xathomatous papules/nodules scattered on the back was done. Histologically, the lesion consisted of dense infiltration of foamy histiocytes and some Tuton type giant cells associated with a sclerotic process. In constrast to the previous biopsy of the skin, which was diagnosed as LCH, there was no nuclear atypia and mitotic activity. Immunohistochemically, the foamy histiocytes were positive for CD68 and negative for CD1a and S-100 protein.