Asano Tomoyuki, Suzutani Ken, Watanabe Aya, Honda Aki, Mori Natsumi, Yashiro Makiko, Sato Shuzo, Kobayashi Hiroko, Watanabe Hiroshi, Hazama Momoko, Kanno Takashi, Suzuki Eiji, Ishii Shiro, Migita Kiyoshi
Department of Rheumatology Department of Dermatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima Department of Rheumatology, Ohta Nishinouchi General Hospital Foundation, Nishinouchi, Koriyama Department of Radiology, Fukushima Medical University School of Medicine, Hikarigaoka, Fukushima, Fukushima, Japan.
Medicine (Baltimore). 2018 Aug;97(33):e11449. doi: 10.1097/MD.0000000000011449.
Multicentric reticulohistiocytosis (MRH) is a rare histiocytic disorder that involves the skin, joints, and visceral organs.
We report a 67-year-old woman with MRH who presented with a 2-years history of polyarthralgia and skin nodules. Her symptoms were an inflammatory polyarthropathy with punched-out lesions of the distal interphalangeal (DIP) joints of both hands. Doppler ultrasonography of the hands showed large bone erosions with power Doppler signals in the DIP joints. F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) demonstrated increased FDG uptake in cutaneous papules surrounding the affected joints, suggesting an inflammatory process. There was no evidence of malignancy. Biopsy samples of skin nodules exhibited dermal infiltration with CD68-positive histiocytes and multinucleated giant cells. The patient was diagnosed with MRH and treated with combination therapy comprising a steroid (prednisolone), tacrolimus, methotrexate, and infliximab, which resulted in clinical improvement. Following infliximab treatment, there was a significant decrease in a bone resorption marker (tartrate-resistant acid phosphatase 5b: TRACP-5b), suggesting that tumor necrosis factor-α targeting therapy may inhibit osteoclast formation and resorption activity in patients with MRH.
MRH is a progressive destructive arthritic condition, and early diagnostic and therapeutic strategies are necessary to improve the outcome. FDG-PET/CT and joint ultrasonography might be noninvasive imaging modalities that could help diagnose MRH.
多中心网状组织细胞增生症（MRH）是一种罕见的组织细胞疾病，累及皮肤、关节和内脏器官。
我们报告一名67岁患有MRH的女性，她有2年的多关节痛和皮肤结节病史。她的症状是炎症性多关节病，双手远端指间关节（DIP）有穿凿样损害。手部多普勒超声显示DIP关节有大的骨质侵蚀及能量多普勒信号。F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描（FDG-PET/CT）显示受累关节周围皮肤丘疹处FDG摄取增加，提示有炎症过程。无恶性肿瘤证据。皮肤结节活检样本显示真皮有CD68阳性组织细胞和多核巨细胞浸润。该患者被诊断为MRH，并接受了包括类固醇（泼尼松龙）、他克莫司、甲氨蝶呤和英夫利昔单抗的联合治疗，临床症状改善。英夫利昔单抗治疗后，骨吸收标志物（抗酒石酸酸性磷酸酶5b：TRACP-5b）显著下降，提示肿瘤坏死因子-α靶向治疗可能抑制MRH患者破骨细胞的形成和吸收活性。
MRH是一种进行性破坏性关节炎疾病，早期诊断和治疗策略对于改善预后很有必要。FDG-PET/CT和关节超声可能是有助于诊断MRH的非侵入性成像方式。
