An intriguing disease with unusual presentation: A Rosai-Dorfman’s case report

Abstract

Rosai-Dorfman disease is a rare non-Langerhans histiocytic disease characterized by lymph node enlargement and extra-nodal involvement. Extranodal disease occurs in 40% of cases and common sites of involvement are: skin, nasal cavity, bone, orbital tissue and central nervous system. Lesions are usually plaques, nodules or papules of insidious growth and no associated symptoms. We report here an atypical case of Rosai Dorfman in an adolescent with fever of undetermined origin, without significant lymphadenopathy and diffuse facial edema.