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Orbital Rosai-Dorfman disease: a literature review
Scott F McClellan1, Darryl J Ainbinder
1Bayne-Jones Army Community Hospital, Ophthalmology Clinic, Fort Polk , Louisiana 71459 , USA and.
Introduction:
Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a predilection for the head and neck. Isolated orbital involvement without systemic clinical features is very uncommon.
Background:
Classic histopathologic features include phagocytized but intact lymphocytes within large histiocytes; a conditioned termed emperipolesis. Immunohistochemical stains are strongly positive for S-100 and CD68 but negative for CD1a in most cases. Orbital cases are often amenable to complete surgical resection.
Conclusion:
RDD should be considered in the differential diagnosis of a young patient in good health who presents with a marked but benign enlargement of an orbital mass. A review of the literature regarding this disease entity and its orbital manifestations is discussed.
