Rosai-Dorfman disease: A rare clinico-pathological presentation

Abstract

Rosai-Dorfman disease (RDD) is also known as sinus histiocytosis with massive lymphadenopathy (SHML). It is a rare benign, self-limiting, idiopathic proliferative disease of phagocytic histiocytes affecting young age group presenting with massive painless cervical lymphadenopathy. It has nodal a also extra-nodal involvement with episodes of exacerbation a remissions, whose etiology remains poorly elucidated. It's highly variable in its clinical presentation a response to treatment. Its treatment is poorly defined but the prognosis is usually favorable. Here we are reporting a rare unusual clinical presentation of cervical lymphadenopathy and nasal mass diagnosed as RDD with cyto-histopathological correlation. Only few such cases have been reported in literature. We also emphasize that the clinicians and pathologists should always be aware of Rosai-Dorfman disease in making a differential diagnosis of cervical lymphadenopathy. Keywords: Rosai-Dorfman Disease, Cervical lymphadenopathy, Histiocytes, Emperipolesis.