Melkersson-Rosenthal syndrome

Otolaryngol Head Neck Surg. 2008 Feb;138(2):246-51. doi: 10.1016/j.otohns.2007.11.015.

Abstract

Objectives: To study characteristics of Melkersson-Rosenthal syndrome (MRS) patients with facial palsy (FP) and differences in patients treated at the Departments of Otorhinolaryngology and Dermatology.

Methods: Clinical picture of MRS was studied from patient charts at two departments. Patients with FP received a questionnaire and were examined. Tissue biopsies were searched for non-necrotizing granulomatous infiltrations typical of MRS and blood DNA for UNC-93B1 gene mutations predisposing to herpesvirus infection.

Results: At the Department of Otorhinolaryngology, all 18 MRS patients had FP, 9 the triad form. Two patients revealed non-necrotizing granulomatous infiltrations during acute edema episodes; another two had association with uveitis. Edema was rarely persistent and did not dominate the clinical picture. No UNC-93B1 mutations were found. At the Department of Dermatology, 2 patients had triad MRS and 15 had monosymptomatic granulomatous cheilitis with persistent edema and typical MRS histology.

Conclusion: The clinical picture of MRS with FP differed from the current knowledge of edema-dominated MRS. More studies focusing on MRS with FP would broaden our understanding of the syndrome.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Biopsy
  • DNA / genetics
  • Diagnosis, Differential
  • Follow-Up Studies
  • Humans
  • Melkersson-Rosenthal Syndrome / diagnosis*
  • Melkersson-Rosenthal Syndrome / etiology
  • Membrane Transport Proteins / genetics
  • Middle Aged
  • Mutation
  • Polymerase Chain Reaction
  • Retrospective Studies
  • Severity of Illness Index
  • Surveys and Questionnaires

Substances

  • Membrane Transport Proteins
  • UNC93B1 protein, human
  • DNA