Melkersson-Rosenthal Syndrome Presenting as Chronic Eyelid Swelling.

Abstract

Melkersson-Rosenthal Syndrome (MRS) is a rare disorder characterized by orofacial edema, facial palsy, and fissured tongue. A 64-year-old man presented with a fissured tongue and persistent chronic right upper eyelid edema of 15 years duration. The diagnostic biopsy revealed non-necrotizing granulomatous inflammation adjacent to blood and lymphatic vessels. Characteristic granulomas with focal occlusion of dilated lymphatic channels were present. Special stains for fungi, acid-fast microorganisms and bacteria were negative. Ptosis due to a mechanical effect of the persistent eyelid edema required surgical treatment including debulking and advancement of the levator muscle. At 6 months after surgery the patient showed symmetrical eyelid position without edema. The case demonstrates an uncommon presentation of Melkersson-Rosenthal syndrome with prominent upper eyelid edema and lingua plicata, which was initially misdiagnosed. The clinical findings coupled with the characteristic granulomatous lymphangitis present in the biopsy are crucial for a definite diagnosis.