Melkersson-Rosenthal syndrome and its variants- The Mayo Clinic experience

Abstract

RATIONALE: Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis are conditions in which patients develop swelling of different parts of the face secondary to non-caseating granulomas. We examined the clinical presentation, therapeutic intervention, and coexisting disorders in those patients. METHODS: The Mayo Clinic database was reviewed for the diagnoses of Melkersson-Rosenthal syndrome, granulomatous cheilitis or orofacial granulomatosis between 1976 and 2009. We reviewed the clinical presentation, laboratory studies and treatment regimens. RESULTS: There were a total of 72 patients who presented with focal facial swelling and had area- specific biopsies that showed non- caseating granulomas consistent with Melkersson-Rosenthal syndrome. There were 51 females and 21 males with a median age of 39 years (range 8 to79 years). The complete triad of Melkersson- Rosenthal Syndrome, which includes orofacial edema, intermittent facial palsy and lingua plicata, was present in seven patients (10%). Orofacial edema was present in all the patients with the lips being the most common area affected (74%). Lingua plicata and facial palsy were present in 47% and 10% of the patients, respectively. Crohn's disease was present in 8% of the patients. None of the patients had sarcoidosis. Two patients had systemic lupus. Only two of the fourteen patients had improvement with antihistamines. About half of those who received oral steroids (n= 25) and about 70% of those who received intralesional steroids (n= 17) had an improvement. Other successful therapies included dapsone, minocycline, or metronidazole. Two patients had cheiloplasty. CONCLUSIONS: In patients with Melkersson-Rosenthal syndrome, oral or intralesional steroids are a management option.