Paire V, Bossard C, Vabres B, Weber M, Péchereau A
Service d'ophtalmologie, Hôtel Dieu, Centre Hospitalier Universitaire, Nantes, France.
J Fr Ophtalmol. 2008 Sep;31(7):673-82. doi: 10.1016/s0181-5512(08)74381-x.
Rosai-Dorfman disease (or sinus histiocytosis with massive lymphadenopathy) is a rare, benign entity, characterized by a histiocytic proliferative disorder that affects mainly the lymph node sinuses but also the lymphatics in extranodal manifestations. It affects mainly young men. The clinical syndrome consists of adenopathies, notably cervical, with fever and extranodal manifestations. Ophthalmological involvement is not frequent (10%), concerning above all the orbits, eyelids, and lacrimal glands, but cornea or uveal tissue can also be affected. Definitive diagnosis is anatomopathological, showing a histiocytic proliferation with lymphophagocytosis (emperipolesis) whose immunohistochemical analysis reveals CD68+ and PS100+.
We describe a consecutive series of three cases seen in 2006 at the University Hospital of Nantes (France). One case presented an intraconical location with exophthalmos. The other two showed lacrimal fossa involvement.
Our series, through the patients' mean age (61 years), symptom progression (from a few weeks to several years), clinical variability (several extranodal sites with no lymphadenopathy), and anatomopathological problems (especially extemporaneous analysis) shows the pathology's polymorphism as well as the diagnosis and therapeutic problems.
A rare and unrecognized entity, Rosai-Dorfman disease can affect the entire ocular globe. Definitive diagnosis is only established by anatomopathological study. Although it is benign, ophthalmological involvement can cause severe damage, which requires efficacious treatment and a multidisciplinary approach.
罗萨伊 - 多夫曼病（或伴有巨大淋巴结病的窦性组织细胞增多症）是一种罕见的良性疾病，其特征为组织细胞增生性疾病，主要影响淋巴结窦，但在结外表现中也会累及淋巴管。该病主要影响年轻男性。临床综合征包括淋巴结肿大，尤其是颈部淋巴结肿大，伴有发热和结外表现。眼部受累并不常见（10%），主要涉及眼眶、眼睑和泪腺，但角膜或葡萄膜组织也可能受到影响。确诊依靠解剖病理学检查，表现为伴有淋巴细胞吞噬现象（血细胞吞噬作用）的组织细胞增生，其免疫组化分析显示CD68 +和PS100 +。
我们描述了2006年在法国南特大学医院连续观察到的三例病例。一例表现为锥内占位并伴有眼球突出。另外两例显示泪窝受累。
我们的病例系列通过患者的平均年龄（61岁）、症状进展（从数周至数年）、临床变异性（多个结外部位且无淋巴结肿大）以及解剖病理学问题（尤其是即时分析），显示了该疾病的多态性以及诊断和治疗方面的问题。
罗萨伊 - 多夫曼病是一种罕见且未被充分认识的疾病，可累及整个眼球。只有通过解剖病理学研究才能确诊。尽管它是良性的，但眼部受累可导致严重损害，这需要有效的治疗和多学科方法。
