Abstract :
We report a case of 17-year old male who came to us with complaints of recurrent bilateral diffuse soft tissue swellings in the anterior orbit. He also had a swelling over the left parotid region and bilateral submandibular lymphadenopathy. Patient had several systemic associations such as congenital deafness, polyarthritis, hypoparathyroidism, pigmented hypertrichosis and juvenile onset Diabetes mellitus, which lead of the clinical diagnosis of Rosai Dorfman Syndrome (RDS). Excision biopsy of the masses was done and histopathological examination revealed “emperipolesis” – engulfment of lymphocytes by histiocytes, characteristic of RDS. Rosai-Dorfman Syndrome (RDS) is a rare benign, idiopathic proliferative histiocytic disorder presenting with a spectrum of systemic associations, most of which manifested in our patient. Diagnosis was made clinically first, and confirmed by histopathology later.
Keyword :
Emperipolesis, Rosai Dorfman Syndrome, Orbit