Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML)(Rosai Dorfman disease) is a rare disorder of unknown etiology, usually associated with lymph node enlargement in various supercial or deep sites. These cases can often be misdiagnosed as lymphoma. The disease affects predominantly the younger age group. It is a benign proliferative histiocytic disorder morphologically characterized by 'emperipolesis'. Extranodal involvement isnot rare (43%).Sites include skin, nasal cavity, para nasal sinuses, eyelids, orbit, bone and central nervous system (1)

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