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J Zhejiang Univ (Med Sci)  2021, Vol. 50 Issue (2): 148-154    DOI: 10.3724/zdxbyxb-2021-0103
    
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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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 wordsMelkersson-Rosenthal syndrome      Rare diseases      Etiology      Pathology      Diagnosis      Therapy      Review     
Received: 25 January 2021      Published: 18 June 2021
CLC:  R781.5  
Corresponding Authors: ZENG Xin     E-mail: wendy_k0@163.com;zengxin22@163.com
Cite this article:

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

URL:

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


梅–罗综合征患者的表征及其诊治

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 
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