Rosai-Dorfman Disease Presenting with Recurrent, Unilateral Submandibular Lymph Adenitis: a Case Report

Rosai-Dorfman Disease (RDD) is a rare disease of unknown aetiology, initially described as a separate entity in 1969 by Rosai and Dorfman. We describe here a case of RDD presenting with acute, recurrent lymphadenitis and massive lymphadenopathy. The patient's good response to the antibiotics was a false pointer towards infective pathology. The repeated fine needle aspiration cytology was inconclusive or suggested nonspecific reactive hyperplasia. The excision biopsy confirmed the diagnosis of RDD and was supported by immune-histochemistry for S-100 antigen. No other lymph node group was involved clinically or radiologically. The patient was treated with long-term antibiotic and low-dose predenisolone. The residual lymph node mass was excised after 3 months of first surgery. The patient is on regular follow-up for last 2 years and no recurrence has been reported.