Rosai Dorfman Disease: A Rare Cause of Lymphadenopathy

Rosai-Dorfman disease (RDD) is a rare cause of generalized lymphadenopathy due to sinus histiocytosis. This is often confused with infective or malignant aetiologies. We here report a case of a 46 year old female from Eastern India presenting with progressive generalized lymphadenopathy for four years. She had no systemic symptoms like fever. All blood tests including viral serologies were normal. Excision biopsy showed lymph node sinus infiltration with histiocytes and emperipolesis. The histiocytes were CD68 positive and CD1a negative. Thus the case was pathologically diagnosed as RDD. The patient responded to oral prednisolone (1 mg/Kg). There was no extranodal involvement. The relevant literature regarding RDD has also been discussed at length.