A Case of Melkersson-Rosenthal syndrome

ABSTRACT

Melkersson-Rosenthal syndrome (MRS) is a rare form of hereditary angioedema characterized by a triad of orofacial swelling, relapsing facial paralysis, and a fissured tongue. However, the classic triad is not frequently seen in its complete form, and monosymptomatic or oligosymptomatic forms are more common. Case A 20-year-old man presented with recurrent labial swelling 9 months ago and recently with a fissured tongue. The surface of the tongue showed deep furrows characteristic of lingua plicata, and an edematous enlargement was observed on the lower lips. Upon neurologic examination, a left sided facial palsy of a peripheral type was noted without abnormalities in the taste sense or lacrimation. An electromyography of the left frontal muscle showed positive sharp waves and fibrillation potentials. Biopsies performed on the lower lips revealed the congested vessels and perivascular inflammatory cells. We report a 20-year-old man with a classical triad of symptoms of Melkersson-Rosenthal syndrome.