Melkersson-Rosenthal Syndrome (MRS) is considered very rare with a estimated prevalence rate of 0.08%, but is likely under diagnosed. Awareness of MRS is important to avoid unnecessary repeated work ups, and for prompt treatment.

Melkersson-Rosenthal Syndrome  is a rare granulomatous inflammatory disease of unilateral facial nerve palsy, orofacial edema, and fissured tongue. The triad occurs in 8% - 25% of the cases. Monosymptomatic cases are more common. Trigeminal nerve and stapedius involvement in association with MRS has been reported. The etiology is still unclear.  Biopsy of swollen mucocutaneous tissue shows non-caseating granulomatous inflammation. Association of MRS with mycobacterium tuberculosis, viral infections, genetic predisposition, and links to Crohn’s and Sarcoidosis has been speculated. MRS is usually recurrent with low probability of complete resolution. However Stein et al reported a case of MRS treated with TNF alpha inhibitors ( Adalimumab) with complete  remission for 8 years.

A 36-year-old female developed a right facial weakness, numbness, hyperacusis, and right upper lip swelling in December of 2018. On examination patient had fissured tongue (lingua plicata).10 years ago patient had right facial weakness with lower lip swelling. Biopsy of the lower lip confirmed the diagnosis of MRS

MRI of the brain, MRA and MRV of the head without contrast  was normal. On oral prednisone patient’s right sided  facial weakness resolved. 2 weeks later she developed  right sided hearing loss. She received 10 more days of oral steroid but upper lip swelling and hearing impairment persisted.

We present a patient with 2 episodes of  MRS, 10 years apart. During the first episode symptoms completely resolved and the patient was in remission for 10 years.  During recent episode she has residual right upper lip swelling and hearing impairment inspite of oral steroid treatment . We are considering intralesional steroid and immunomodulatory therapy such as TNF alpha inhibitors (Adalimumab ).