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A Mini-Review of Ocular and Periocular Rosai-Dorfman Disease: A Masquerading Pathology with Unknown Etiology

Babak Masoomian ; Ali Haghbin ; Shahriar Ghodous

Rosai-Dorfman disease (RDD) is a rare sub type of non-Langerhans cell histiocytosis with a predilection for the head and neck that specially involve cervical lymph nodes. Classic histopathologic features include emperipolesis and Immunohistochemical stains are strongly positive for S-100 and CD68 but negative for CD1a in most cases. The exact nature and RDD etiology remains unknown. More than 40% of RDD patients show extra nodal involvements. Ophthalmic manifestations can be detect in 10% of all patients in which, the highest prevalence is related to orbital lesions. In the presence of orbital and intraocular lesions, systemic involvements are more likely to be associated. There is no specific treatment for this disease therefore, treatment modalities should be individualize based on site of involvement and presence or absence of systemic involvement.