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Rare diagnosis of Melkersson-Rosenthal syndrome in a paediatric patient
  1. Anuradha Pavuluri1,2,
  2. Stephanie Smith1 and
  3. Umesh Narsinghani1,2
  1. 1Pediatrics, Medical Center Navicent Health, Macon, Georgia, USA
  2. 2Pediatrics, Mercer University, Macon, Georgia, USA
  1. Correspondence to Dr Anuradha Pavuluri; anuradha.pavuluri{at}atriumhealth.org

Abstract

Melkersson-Rosenthal syndrome (MRS) is a rare neurocutaneous syndrome characterised by the triad of recurrent orofacial swelling, facial nerve palsy and fissured tongue. This diagnosis is particularly rare in children. We aim to increase awareness of the syndromic association of these clinical features since most patients present with a monosymptomatic form, reiterating the importance of detailed history and thorough physical examination, for the timely identification of these patients. Not only the recurring of symptoms, but also the association of MRS with other medical conditions, make ‘earlier’ diagnosis of the Syndrome beneficial. The average delay in diagnosis is 4–9 years. Although most cases resolve without treatment, when treated,steroids are most commonly used. Variable options have been tried for refractory and frequently recurrent cases. We present a case of MRS in a 12-year-old girl, diagnosed 3 years after onset of symptoms. We reviewed updated literature for MRS and associated clinical conditions as well as published treatment options.

  • dermatology
  • ear
  • nose and throat/otolaryngology
  • cranial nerves
  • immunology

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Footnotes

  • Twitter @PavuluriA

  • Contributors AP researched, drafted and edited the report. SS and UN managed the patient and researched and edited the report.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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