Neurological Surgery 脳神経外科39巻12号

2011年12月発行

症例

成人発症の単発型眼窩部ランゲルハンス細胞組織球症の1例

著者： 神宮字伸哉¹ 尾山徳秀² 米岡有一郎¹ 福多真史¹ 藤井幸彦¹

所属機関： ¹新潟大学脳研究所脳神経外科 ²新潟大学眼科

ページ範囲：P.1183 - P.1188

Ⅰ．はじめに

　ランゲルハンス細胞組織球症（Langerhans cell histiocytosis：LCH）は，以前はhistiocytosis X，eosinophilic granuloma，Letterer-Siwe病，Hand-Schüller-Christian病などと呼ばれた疾患の総称である．主に1～3歳に好発し，小児における年間発症率は100万人に5人前後とする報告が多く^2,13)，成人発症例はさらに少ないとされる⁹⁾．病的なランゲルハンス細胞が異常増殖する非腫瘍性の疾患で，全身に病変が生じ得る可能性があるが，骨病変の頻度が最も高く，なかでも頭蓋骨に生じることが多い⁹⁾．現在では，罹患する臓器と病変の数により，単一臓器単発（single-system single-site：SS）型，単一臓器多発（single-system multi-site：SM）型，多臓器病変（multi-system multi-site：MM）型と分類される．

　今回，われわれは成人発症の眼窩に発生したLCHを経験したので報告する．

参考文献

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