Patel Maharshi H, Jambhekar Kedar R, Pandey Tarun, Ram Roopa
Department of Radiology, Musculoskeletal and Body MRI Division, University of Arkansas for Medical Sciences, Arkansas, USA.
Indian J Radiol Imaging. 2015 Jul-Sep;25(3):284-7. doi: 10.4103/0971-3026.161459.
Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a non-neoplastic condition which typically presents as massive, bilateral cervical lymphadenopathy and can involve multiple extranodal organ systems such as skin, eyes, and upper respiratory tract in about 28% cases. Bone lesions in association with nodal disease are seen in less than 10% cases. Isolated bone involvement as the only manifestation of SHML is extremely rare, with less than 50 cases reported in the literature. We report a very uncommon case of Rosai-Dorfman disease with isolated multifocal osseous involvement as the only presenting feature, involving about 10 different sites with no lymphadenopathy or other organ system involvement.
伴巨大淋巴结病的窦性组织细胞增生症(SHML)或罗萨伊-多夫曼病是一种非肿瘤性疾病,通常表现为双侧巨大颈部淋巴结病,约28%的病例可累及多个结外器官系统,如皮肤、眼睛和上呼吸道。与淋巴结疾病相关的骨病变见于不到10%的病例。孤立性骨受累作为SHML的唯一表现极为罕见,文献报道不足50例。我们报告了一例非常罕见的罗萨伊-多夫曼病病例,其唯一的表现为孤立性多灶性骨受累,累及约10个不同部位,无淋巴结病或其他器官系统受累。