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Melkersson-Rosenthal syndrome

Disease definition

A rare orofacial granulomatosis characterized by the triad of recurrent or persistent orofacial edema (facial and lip edemas), fissured tongue, and relapsing, unilateral or bilateral peripheral facial nerve paralysis. Most cases present with partial symptoms. Typical age of onset is in childhood or adolescence. Histological examination shows non-caseating epithelioid cell granulomas and lymphedema.

ORPHA:2483

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Childhood
  • ICD-10: G51.2
  • OMIM: 155900
  • UMLS: C0025235
  • MeSH: D008556
  • GARD: 7010
  • MedDRA: 10027166
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