AI Article Synopsis

  • Melkersson-Rosenthal syndrome (MRS) is a rare condition characterized by orofacial swelling, facial nerve palsy, and a fissured tongue, with most cases presenting in a monosymptomatic form, especially in children.
  • Awareness of MRS is crucial since the average diagnosis delay is 4-9 years, and earlier identification can help manage associated health issues.
  • While many cases may resolve on their own, treatment typically involves steroids, and various options have been explored for cases that are severe or recurrent.

Article 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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8201983PMC
http://dx.doi.org/10.1136/bcr-2020-238431DOI Listing

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