[Orbital locations in Rosai-Dorfman disease: a series of three consecutive cases]

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.