Rosai-Dorfman disease in a 12-year-old Nigerian male

Abstract

Rosai-Dorfman disease is a rare disorder of systemic histioproliferation, first described in 1969 by Juan Rosai and Ronald Dorfman. It is characterised by massive lymphadenopathy, particularly of the head and neck region, and it is sometimes associated with extranodal involvement. This is the first case to be reported from our centre. We report a case of Rosai-Dorfman disease in a 12-year-old Nigerian boy who presented at the children’s outpatient clinic of the Federal Medical Centre, Owerri, Imo State, Nigeria, with a 5-year history of bilateral neck swelling, a 2-year history of groin swelling and a recurrent fever of over a year’s duration. An initial diagnosis of Hodgkin’s lymphoma was made. An excisional biopsy of the cervical lymph node was undertaken and the histology showed massive histiocytosis with emperipolesis, which confirmed the diagnosis of Rosai-Dorfman disease.Rosai-Dorfman disease, although a rare clinical entity, should be suspected in patients presenting with massive lymphadenopathy located especially in the neck and groin regions.