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浙江大学学报（医学版）
浙江大学学报（医学版）  2021, Vol. 50 Issue (2): 148-154    DOI: 10.3724/zdxbyxb-2021-0103
专题报道     
梅–罗综合征患者的表征及其诊治
旷文静(),罗小波,王冏珂,曾昕()
四川大学华西口腔医院口腔黏膜病科 口腔疾病研究国家重点实验室 国家口腔疾病 　临床医学研究中心，四川 成都 610041
Research progress on Melkersson-Rosenthal syndrome
KUANG Wenjing(),LUO Xiaobo,WANG Jiongke,ZENG Xin()
Department of Oral Medicine, West China Hospital of Stomatology, Sichuan University, State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, Chengdu 610041, China
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摘要：

梅–罗综合征（MRS）是一种罕见的累及神经、皮肤、黏膜的综合征，以复发性口面部肿胀、复发性面瘫和裂纹舌三联征为特征，好发于青壮年。MRS 病因尚不明确，可能与微生物因素、免疫因素、遗传因素等有关，近年关于遗传因素致病的研究较受关注。MRS 的治疗主要包括药物治疗和手术治疗，其中药物治疗首选糖皮质激素，但具体用法用量、治疗效果尚无定论；唇部肿胀较严重者可行唇成形手术改善唇部外观。本文就 MRS 流行病学现状、病因、组织病理学表现、临床表现和分型、诊断、鉴别诊断、治疗方面的研究进展进行综述，旨在增加临床医生对该病的认识。
关键词： 梅–罗综合征;  罕见病;  病因;  病理学;  诊断;  治疗;  综述    
Abstract:

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-muco-cutaneous syndrome, which is characterized by recurrent orofacial swelling, recurrent facial paralysis and fissured tongue. It has a high prevalence in young adults. Up to now, the etiology of MRS is still not clear, it may related to infection, immune deficiency and hereditary factors. The pharmacological therapy and surgery are the main treatment. Corticosteroids seems to be the drug of choice for MRS patient, but the specific dosage and therapeutic effect have not yet been determined. Surgeries of lips provide excellent results in persistentlip edema MRS cases. This article reviews the research progress on MRS, focusing on its epidemiology, etiology, histopathological characteristics, clinical manifestations, classification, diagnostic criteria, differential diagnosis and treatment, to provide information for its early diagnosis and appropriate treatment.
Key words: Melkersson-Rosenthal syndrome    Rare diseases    Etiology    Pathology    Diagnosis    Therapy    Review
收稿日期: 2021-01-25 出版日期: 2021-06-18
CLC:  R781.5  
基金资助: 中国医学科学临床与转化医学研究基金（2020-I2M-C&T-A-023）；国家自然科学基金（81771081）
通讯作者: 曾昕     E-mail: wendy_k0@163.com;zengxin22@163.com
作者简介: 旷文静，硕士研究生，主要从事口腔黏膜病的病因及防治研究；E-mail：wendy_k0@163.com；https：//orcid.org/0000-0002-0662-9614
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引用本文:

旷文静,罗小波,王冏珂,曾昕. 梅–罗综合征患者的表征及其诊治[J]. 浙江大学学报（医学版）, 2021, 50(2): 148-154.

KUANG Wenjing,LUO Xiaobo,WANG Jiongke,ZENG Xin. Research progress on Melkersson-Rosenthal syndrome. J Zhejiang Univ (Med Sci), 2021, 50(2): 148-154.

链接本文:

http://www.zjujournals.com/med/CN/10.3724/zdxbyxb-2021-0103    或    http://www.zjujournals.com/med/CN/Y2021/V50/I2/148

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