Scott F McClellan1, Darryl J Ainbinder. 1. Bayne-Jones Army Community Hospital, Ophthalmology Clinic, Fort Polk , Louisiana 71459 , USA and.
Abstract
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.
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.