Journal of Research in Medical Sciences1735-199516920110915Rosai-Dorfman disease with nodal and extranodal involvements: A case report67816781ENMehriNajafi-SaniHosseinSaneianAssistant Professor of Pediatric Gastroenterology, Department of Pediatrics, Isfahan University of Medical Sciences, Isfahan, Iran. saneian@med.mui.ac.irFatemehMahjoub201102102011082920110630Rosai-Dorfman disease (RDD) is a rare lymphoproliferative disorder with nodal and extranodal involvements. Here we report a case of RDD in a 15-year-old female who presented with epigastric pain, fatigue, Raynaud phenomenon in fingers, submandibular lymphadenopathy, proptosis, hepatosplenomegaly, and round shape painless patches on the extensor surfaces. Histological examination of the submandibular lymph nodes and skin biopsy demonstrated evidences of RDD. Patient was treated with prednisone and thereafter, with azathioprine. After one year, prednisone was discontinued and all of the symptoms and signs, except proptosis, were resolved. This report highlights the extranodal manifestations of RDD. The presentation, differential diagnosis, and treatment are discussed. KEYWORDS: Rosai-Dorfman Disease, Sinus Histiocytosis, Lymphadenopathy.