結膜下腫瘍と視神経乳頭腫脹を伴ったRosai-Dorfman病の1例

A case of Rosai-Dorfman disease with subconjunctival tumor and optic disc swelling

要約　背景：Rosai-Dorfman病は原因不明の良性の組織球増殖性疾患で，sinus histiocytosis with massive lymphadenopathyともいう。目的：眼症状を伴ったRosai-Dorfman病の1例の報告。症例：47歳男性が2年前からの両眼の視力低下で受診した。矯正視力は右0.06，左0.3で，右眼に鮭紅色の結膜下腫瘤，両眼に軽度の虹彩毛様体炎，視神経乳頭腫脹，黄斑浮腫があった。鼠径リンパ節が腫脹し，下腿に色素斑と皮膚下結節があった。リンパ節の生検でリンパ球を貪食した組織球増殖があり，本病と診断した。プレドニゾロンの全身投与で皮膚と眼病変が軽快した。結論：皮膚病変とリンパ節腫脹を伴う結膜下腫瘤とぶどう膜炎の症例では，Rosai-Dorfman病の可能性がある。

Abstract.　Background：Rosai-Dorfman disease is also called as sinus histiocytosis with massive lymphadenopathy and is characterized by benign proliferation of histiocytes. Purpose：To report a case of Rosai-Dorfman disease with ocular manifestations. Case：A 47-year-old male presented with progressive impairment of vision in both eyes since 2 years before. Corrected visual acuity was 0.06 right and 0.3 left. The right eye showed a pink-colored subconjunctival tumor. Both eyes showed mild iritis,macular edema and swelling of optic disc. He showed swelling of inguinal lymphnodes with pigment patches and subcutaneous nodules in the lower extremities. Biopsy of lymphnodes showed proliferation of histiocytes containing phagocytosed lymphocytes,leading to the diagnosis of Rosai-Dorfman disease. Conclusion：Rosai-Dorfman disease is a possibility in a case of subconjunctival tumor and uveitis with skin and lymphnode lesions.