Najafi-Sani Mehri, Saneian Hossein, Mahjoub Fatemeh
Associate Professor of Pediatric Gastroenterology, Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran.
J Res Med Sci. 2011 Sep;16(9):1251-6.
Rosai-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.
罗萨伊-多夫曼病（RDD）是一种罕见的淋巴增殖性疾病，可累及淋巴结和结外部位。本文报告一例15岁女性RDD患者，该患者表现为上腹部疼痛、疲劳、手指雷诺现象、颌下淋巴结肿大、眼球突出、肝脾肿大以及伸肌表面圆形无痛性斑块。颌下淋巴结组织学检查及皮肤活检证实为RDD。患者先接受泼尼松治疗，之后加用硫唑嘌呤。一年后停用泼尼松，除眼球突出外，所有症状和体征均消失。本报告强调了RDD的结外表现。并对其临床表现、鉴别诊断及治疗进行了讨论。
