Research progress on Melkersson-Rosenthal syndrome

Zhejiang Da Xue Xue Bao Yi Xue Ban. 2021 Apr 25;50(2):148-154. doi: 10.3724/zdxbyxb-2021-0103.

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.

Keywords: Diagnosis; Etiology; Melkersson-Rosenthal syndrome; Pathology; Rare diseases; Review; Therapy.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones
  • Diagnosis, Differential
  • Humans
  • Lip
  • Melkersson-Rosenthal Syndrome* / diagnosis
  • Melkersson-Rosenthal Syndrome* / drug therapy
  • Skin
  • Young Adult

Substances

  • Adrenal Cortex Hormones

Grants and funding

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)