Melkersson-Rosenthal syndrome revisited as a misdiagnosed disease

Am J Otolaryngol. 2009 Jan-Feb;30(1):33-7. doi: 10.1016/j.amjoto.2008.02.004. Epub 2008 Jul 22.

Abstract

Purpose: We aimed to attract our college's attention to the Melkersson-Rosenthal syndrome (MRS), which has been an infrequently encountered subject in otolaryngology journals during the last 10 years.

Materials and methods: A retrospective review of the last 10 years' patient database was performed to find patients with MRS. The medical files, treatment charts, and radiological and histopathological records of these patients were reviewed.

Results: The study group consisted of 3 MRS patients who had been misdiagnosed for 9, 10, and 16 years. Two of them have had the symptoms since adolescence. All of them presented orofacial edema and fissured tongue, whereas first two also had recurrent facial paralysis. Characteristic histopathological features were noted in 1 patient. Electromyography (EMG) was done in 1 patient who underwent facial decompressiom. All patients responded to either systemic or intralesional corticosteroid treatment.

Conclusions: In the daily practice of an otolaryngologist, it is not usual to diagnose a patient as having MRS. We consider that this is partly because of misdiagnosis. We therefore believe that this study will supply an additional aspect to otolaryngologists, in the scope of recurrent facial paralysis and orofacial edema in both children and adults.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Age of Onset
  • Biopsy, Needle
  • Cohort Studies
  • Diagnosis, Differential
  • Diagnostic Errors*
  • Electromyography
  • Facial Paralysis / diagnosis*
  • Facial Paralysis / epidemiology
  • Facial Paralysis / pathology
  • Female
  • Humans
  • Immunohistochemistry
  • Incidence
  • Male
  • Melkersson-Rosenthal Syndrome / diagnosis*
  • Melkersson-Rosenthal Syndrome / drug therapy
  • Melkersson-Rosenthal Syndrome / epidemiology*
  • Melkersson-Rosenthal Syndrome / pathology
  • Prognosis
  • Recurrence
  • Registries
  • Retrospective Studies
  • Severity of Illness Index
  • Tongue, Fissured / diagnosis*
  • Tongue, Fissured / epidemiology
  • Tongue, Fissured / pathology
  • Young Adult

Substances

  • Adrenal Cortex Hormones