頭蓋内病変を伴った全身性黄色肉芽腫症の1例—免疫組織化学的検討

A Case of Systemic Xanthogranulomatosis with an Intracranial Lesion:Immunohistochemical study

I．はじめに

　頭蓋内に黄色腫様病変をきたす疾患は，古くより知られており，これまでにも数多くの報告がある^{1,3-5,7-14）}．その疾患概念や病態のメカニズムについては不明な点が多く，病名そのものについても混乱しているのが現状である^15）．特にHand-Schuller-Christian病などのhis—tiocytosis Xとの鑑別は容易でなく，類縁疾患としてまとめられてきた^3,7,9,10）．われわれは，頭蓋内病変を伴う全身性黄色肉芽腫症の自験例について，免疫組織化学的検討を行い新しい知見を得たので，文献的考察を加えて報告する．

Abstract

The authors describe a case of a 42-year-old woman with systemic xanthogranulomatosis who primarily manifested bilateral intraorbital tumors, generally effecting many regions. These included the intracranial dura mater, the spinal cord, the retroperitoneum, the pericardium, and the mediastinum. This disorder is basically similar to systemic Weber-Christian diseasel except for the absence of subcutaneous panniculitis. Im-munohistochemical study reveals that this clinical entity is different from histiocytosis X because the origin of the foamy cells is not the T-zone histiocyte but the ma-crophage. The treatment of this disease is also dis-cussed.