帕金森病并发大疱类天疱疮、麻痹性肠梗阻1例并文献分析  

Parkinson's disease complicated with bullous pemphigoid and paralytic intestinal obstruction:a case report and literature analysis

作　　者：寇丽[1] 陈志刚 徐晓华[1] 李振东[1] 邓锦凤[1] KOU Li;CHEN Zhigang;XU Xiaohua;LI Zhendong;DENG Jinfeng(Department of Neurology,the Fifth Affiliated Hospital of Sun Yat-sen University,Zhuhai,Guangdong 519000,China)

机构地区：[1]中山大学附属第五医院神经内科,广东珠海519000

出　　处：《中国医学工程》2023年第3期41-47,共7页China Medical Engineering

摘　　要：目的总结和探讨诊断治疗典型帕金森病(PD)合并大疱类天疱疮和麻痹性肠梗阻的临床经验,以期提高临床治疗水平。方法回顾中山大学附属第五医院神经内科治疗的1例PD并发大疱类天疱疮和麻痹性肠梗阻患者的资料,检索复习国内外相关文献,进行研读分析。结果患者男性,72岁,患PD18年,处于PD晚期,具有典型的PD运动运动症状。随着病情进展,逐渐出现开关现象、异动症和多种非运动症状。2017年5月因多发水泡样皮疹并发热入院,诊断为PD合并大疱类天疱疮,经激素冲击治疗、抗感染、增强免疫等治疗以及加强皮肤护理,患者热退,大泡样皮疹控制,出院继续小剂量皮质激素维持治疗,定期门诊随访无复发。2018年5月突发腹胀伴排便困难再入院,诊断PD合并麻痹性肠梗阻,予胃肠负压减压、肛管排气、灌肠及通便等治疗,肠梗阻解除,腹胀消失,2018年9月14日随访未复发。文献复习显示相关报道不多,检索到密切相关文献27篇,其中涉及大疱类天疱疮16篇,肠梗阻7篇。从中筛选出PD并发大疱类天疱疮、麻痹性肠梗阻文献各5篇。报道PD合并大疱类天疱疮75例,发病率约为3%,可导致中毒休克而致患者死亡。用皮质激素治疗效果明显,可降低患者死亡率;合并麻痹性肠梗阻8例,发病率不详,肠梗阻后可导致肠穿孔1例,肠扭转1例,内科治疗6例,手术治疗2例,均治愈。两种并发症涉及不同的易患因素。结论PD并发大疱类天疱疮、麻痹性肠梗阻均为少见危重症,诊疗经验不多。前者主要应用激素治疗,后者进行综合治疗,严重者需考虑手术治疗。临床早期识别,及时恰当处理,均可治愈,有利于改善患者的生存质量。【Objective】To improve the level of clinical treatment of typical parkinson's disease(PD)with bullous pemphigoid and paralytic intestinal obstruction.【Methods】A case of PD complicated with bullous pemphigoid and paralytic intestinal obstruction in our department was retrospectively reviewed.The related literatures at home and abroad were retrieved and analyzed.【Results】1.A 72-year-old male patient with PD 18 years,in the late stage of PD,with typical non-motor symptoms of PD movement.With the progression of the disease,there are progressive switch events,dyskinesia and various non-motor symptoms.Non-motor symptoms include loss of smell,hallucinations,gastrointestinal dysfunction,psoriasis,vesicular rash,and orthostatic hypotension.In May 2017,the patient was admitted to hospital with multiple vesicular rashes and fever.He was diagnosed as PD complicated with bullous pemphigoid.After hormone shock therapy,anti-infection,immune enhancement and skin care,the fever subsided and the bullous rash was controlled.He was discharged from hospital and continued to receive low-dose corticosteroid maintenance therapy.No recurrence was found after long-term follow-up.In May 2018,sudden abdominal distension accompanied by dysentery was re-admitted to the hospital.PD was diagnosed with paralytic intestinal obstruction.The patients were treated with gastrointestinal negative pressure decompression,anal exhaust,enema and defecation.The intestinal obstruction was relieved and the abdominal distension disappeared.No recurrence was found after 1.5 years of follow-up.2.Literature review showed that there were not many related reports,and 27 related literatures were retrieved,16 of which involved bullous pemphigoid and 7 intestinal obstruction.Five papers on PD complicated with bullous pemphigoid and paralytic intestinal obstruction were selected.Seventy-five cases of PD complicated with bullous pemphigoid and 8 cases of paralytic intestinal obstruction were reported.PD is closely related to the pathogenesis of bullous pemph

关 键 词：帕金森病 大疱类天疱疮 麻痹性肠梗阻 诊断 治疗 

分 类 号：R742.5[医药卫生—神经病学与精神病学]