Isolated maxillary rosai dorfmannn disease masquerading as a malignancy

Abstract

Rosai Dorfmann disease (RDD), first described in 1969 by Rosai and Dorfmann, is a rare, benign disorder of unknown etiology. It is a distinct clinopathological entity, typically characterized by non Langerhan histiocytic cell proliferation with massive cervical lymphadenopathy. Axillary, mediastinal and inguinal nodes are affected rarely. Fever, anemia, leucocytosis, hypergamma globulinemia, raised ESR and rarely autoimmune hemolytic anemia may coexist; mimicking differentials like Wegener’s granulomatosis, Langerhans cell histiocytosis, Tuberculosis, Hodgkin’s lymphoma, monocytic leukaemia and rhinoscleromatosis. Hence, a high clinical suspicion with immunopathological analysis confirms the diagnosis, as in this case report.