Asymmetric polyarthritis as an initial presentation of Rosai-Dorfman disease

Abstract

Rosai-Dorfman disease (RDD) is a rare benign reactive lymphoproliferative disorder characterised by a histopathological pattern with sinus histiocytosis and hemophagocytosis. It usually presents with fever, elevated erythrocyte sedimentation rate, cervical lymphadenopathy, other lymph node and extra-nodal site involvement. We present the case of a 25-year-old female patient with polyarthritis mimicking rheumatoid arthritis (RA). When the para-aortic lymph node was biopsied, it showed extensive histiocytic proliferation; some clusters of plasma cells, lymphocytes and rare multinucleated cells were seen, suggesting a diagnosis of RDD. There is nothing in the literature regarding the polyarthritic presentation of the disease. To the best of our knowledge, our patient is the first case of RDD presenting with a clinical picture mimicking atypical seronegative RA. © 2008 Asia Pacific League of Associations for Rheumatology.