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IP International Journal of Ocular Oncology and Oculoplasty


Clinical manifestations of rosai dorfman syndrome – A case report


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Author Details : Chinmayee JT, Kritika Chopra, Dayananda S. Biligi, Sri Ganesh

Volume : 2, Issue : 4, Year : 2016

Article Page : 263-264


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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.

Keywords
: Emperipolesis, Rosai Dorfman Syndrome, Orbit

How to cite : Chinmayee Jt, Chopra K, Biligi D S, Ganesh S, Clinical manifestations of rosai dorfman syndrome – A case report. IP Int J Ocul Oncol Oculoplasty 2016;2(4):263-264

Copyright © 2016 by author(s) and IP Int J Ocul Oncol Oculoplasty. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (CC-BY-NC 4.0) (creativecommons.org)