Li Yanhong, Wang Zhongming, Zhao Yi, Luo Yubin, Xu Wangdong, Marion Tony N, Liu Yi
Department of Rheumatology, West China Hospital, Sichuan University, Chengdu, Sichuan, PR China Department of Microbiology, Immunology, and Biochemistry, The University of Tennessee Health Science Center, Memphis, TN.
Medicine (Baltimore). 2018 Apr;97(16):e0515. doi: 10.1097/MD.0000000000010515.
Behçet disease (BD) is a recurrent vasculitis characterized by oral and genital mucous membrane ulcers, uveitis, and skin lesions but only rarely leg ulcers. To our knowledge, no efficacious therapy has been described for BD patients with complicating, destructive leg ulcers.
Here, We report the case of a 55-year-old woman with generalized erythema nodosum-like, papulopustular lesions, recurrent oral and genital ulcers accompanied with recurrent leg ulcers and trouble walking.
Based upon the patient's clinical feature and positive pathergy test , BD was confirmed.
Conventional immunosuppressive therapy and anti-tumor necrosis factor inhibitors, adalimumab and etanercept, had no demonstrable clinical effect. Mesenchymal stem cell (MSC) injection combined with low-dose prednisone and thalidomide, however, completely ameliorated the ulcers on one leg, significantly improved ulcers on the other leg, and returned normal function to both legs.
The ulcerative lesions remained in remission, and the affected leg functioned normally after 34 months' follow-up.
Our experience suggests that MSC infusion might be a potentially successful therapy for intractable drug-resistant BD patients with concomitant leg ulcer.
白塞病(BD)是一种复发性血管炎,其特征为口腔和生殖器黏膜溃疡、葡萄膜炎及皮肤病变,但腿部溃疡较为罕见。据我们所知,对于合并有破坏性腿部溃疡的白塞病患者,尚无有效的治疗方法。
在此,我们报告一例55岁女性患者,其患有全身性结节性红斑样丘疹脓疱性病变、复发性口腔和生殖器溃疡,并伴有复发性腿部溃疡及行走困难。
根据患者的临床特征及阳性针刺反应试验,确诊为白塞病。
常规免疫抑制治疗以及抗肿瘤坏死因子抑制剂阿达木单抗和依那西普均未显示出明显的临床效果。然而,间充质干细胞(MSC)注射联合小剂量泼尼松和沙利度胺,完全治愈了一条腿上的溃疡,显著改善了另一条腿上的溃疡,并使双腿功能恢复正常。
经过34个月的随访,溃疡性病变仍处于缓解期,患腿功能正常。
我们的经验表明,对于伴有腿部溃疡的难治性耐药白塞病患者,MSC输注可能是一种潜在的成功治疗方法。