Li Dan, You Longfei, Fan Songqing, Tan Lihua
Department of Radiology, The Second Xiangya Hospital of Central South University, Changsha, Hunan Department of Rehabilitation, The Sixth Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong Department of Pathology, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China.
Medicine (Baltimore). 2017 Dec;96(51):e9399. doi: 10.1097/MD.0000000000009399.
Xanthomatosis often accompanies familial hypercholesterolemia. This disease usually occurs in tendons, most commonly located in the Achilles tendon; occasionally it can also be seen in other systems. Although there are previous reports for bilateral hand extensor tendon involvement, to our knowledge there is no report in English literature regarding bilateral hands with small joint synovium presenting as rheumatoid arthritis. Therefore, the case that is presented in this report is unique.
An 18-year-old woman was admitted to our department because she presented with morning stiffness, joint deformation, and swelling in both hands. Computed tomography of the right hand showed soft tissue swelling on multiple small joints, including metacarpophalangeal and proximal interphalangeal joints, but without obvious bone destruction. There was soft tissue swelling around the small joints, which were hypointensities on T1-weighted and hyperintensities on T2-weighted images, not uniformly enhanced appearances on magnetic resonance imaging.
Biopsy from the 3rd metacarpophalangeal joint capsule of the left hand confirmed xanthoma.
She was treated with statin drugs to reduce blood fat.
After 3 months of follow-up, no recurrence or complications were detected regarding a full range of motion remaining of the affected joints.
The young patient with symptoms of small joint synovium involved in both hands and the performance of magnetic resonance imaging similar to rheumatoid arthritis may be suffering from xanthomatosis.
黄瘤病常伴有家族性高胆固醇血症。这种疾病通常发生在肌腱，最常见于跟腱；偶尔也可见于其他系统。虽然之前有双侧手部伸肌腱受累的报道，但据我们所知，英文文献中尚无关于双侧手部小关节滑膜表现为类风湿关节炎的报道。因此，本报告中呈现的病例是独特的。
一名18岁女性因双手出现晨僵、关节变形和肿胀而入住我科。右手计算机断层扫描显示多个小关节软组织肿胀，包括掌指关节和近端指间关节，但无明显骨质破坏。小关节周围有软组织肿胀，在T1加权像上呈低信号，在T2加权像上呈高信号，磁共振成像上强化不均匀。
左手第3掌指关节囊活检证实为黄瘤。
给予他汀类药物降血脂治疗。
随访3个月后，受累关节全范围活动未发现复发或并发症。
双手小关节滑膜受累且磁共振成像表现类似类风湿关节炎的年轻患者可能患有黄瘤病。
