甲泼尼龙联合吗替麦考酚酯治疗口腔黏膜类天疱疮1例报道及文献回顾

Methylprednisolone combined with mycophenolate mofetil for the treatment of oral mucosal pemphigoid:a case report and literature review

目的探讨吗替麦考酚酯(mycophenolate mofetil,MMF)在口腔黏膜类天疱疮中的应用,为临床提供参考。方法报道1例糖皮质激素联合MMF治疗口腔黏膜类天疱疮,结合文献探讨MMF在口腔黏膜相关大疱类疾病中的临床应用。结果1例临床诊断为“口腔黏膜类天疱疮”患者,在单一使用甲泼尼龙(36 mg,qd,晨服)或联合硫酸羟氯喹(0.1 g/次,bid)、沙利度胺胶囊(50 mg,qd,睡前服)等药物治疗后,患者病情控制缓慢且易复发。随即调整治疗方案,即甲泼尼龙(36 mg,qd,晨服)与MMF(0.5 g/次,bid)联合使用,2周后病损愈合控制较理想。甲泼尼龙联合MMF使用8周后,甲泼尼龙逐渐减量至(12 mg,qd),MMF减为(0.5 g,qd),患者症状改善明显,口内未见明显病损,后按照类天疱疮治疗原则进行减量维持,目前已使用甲泼尼龙(8 mg,qd,晨服)以及MMF(0.5 g,qd)维持治疗6个月,仍在随诊中,病情稳定无明显病损及新发水疱,未见明显副作用。文献回顾表明,MMF在皮肤科领域被广泛应用于治疗结缔组织疾病、自身免疫性疱性疾病等多种免疫性疾病。根据MMF不良反应的文献报道,消化系统反应为最为常见,因此有活动性胃肠疾病的患者慎用,其次是骨髓抑制,建议对使用MMF患者进行肝功能及血常规等指标监测,本病例在开始使用糖皮质激素及免疫抑制剂联合治疗后,定期检查血常规及肝功能,未发现明显异常改变。MMF在累及皮肤的类天疱疮治疗上已有相关文献报道证明其安全性和有效性,然而口腔黏膜病医生仍缺乏此药治疗黏膜类天疱疮的相关经验。结论MMF作为一种新型免疫抑制剂,安全性高,无明显副作用,对于临床上病情较重、难治性口腔黏膜类天疱疮患者,可考虑作为联合辅助用药。

Objective To investigate the application of mycophenolate mofetil(MMF)in oral mucosal pemphigoid and provide a clinical reference.Methods One case of glucocorticoids combined with MMF in the treatment of oral mucosal pemphigoid was reported,and the clinical application of MMF in oral mucosarelated bullous diseases was discussed.Results One patient with a clinical diagnosis of“oral mucosal pemphigoid”was treated with methylprednisolone(36 mg,qd,morning dose)or combined hydroxychloroquine sulfate(0.1 g/time,bid)and thalidomide capsules(50 mg,qd,bedtime)and other drugs.The patient’s disease was slowly controlled but prone to recurrence.The treatment regimen was immediately adjusted,i.e.,methylprednisolone(36 mg,qd,morning dose)was combined with MMF(0.5 g/time,bid)for 2 weeks,which resulted in ideal lesion healing control.After 8 weeks of methylprednisolone com bined with MMF,the dose of methylprednisolone was gradually reduced to 12 mg,qd,and MMF was reduced to 0.5 g,qd,the patient’s symptoms improved significantly,and no obvious lesions were found in the mouth.The dose was then reduced and maintained according to the principle of pemphigoid treatment.Methylprednisolone(8 mg,qd,morning dose)and MMF(0.5 g,qd)have been used for 6 months of maintenance treatment,and they are still being followed up.As yet,the patient’s condition is stable without obvious lesions and new blisters,and no obvious side effects have been observed.A review of the literature shows that MMF is widely used in the field of dermatology to treat a variety of im mune diseases,such as connective tissue diseases and autoimmune blistering diseases.According to the reports of ad verse reactions to MMF,digestive system reactions are the most common adverse reactions;therefore,patients with ac tive gastrointestinal diseases should be treated with caution,followed by bone marrow suppression,and it is recommend ed to monitor liver function and blood routine in patients using MMF.The safety and efficacy of MMF for treating pem phigoid involving the skin have been reported in the literature,but oral mucosal doctors still lack experience for treating mucous membrane pemphigoid.Conclusions As a new immunosuppressant,MMF has high safety and no obvious side effects and can be considered as a combination adjuvant drug for patients with severe clinical disease and refractory oral mucosal pemphigoid.