Melkersson-Rosenthal syndrome: a review of 36 patients

The Melkersson-Rosenthal syndrome is an uncommon condition of uncertain pathogenesis and cause. The classic triad of signs includes recurrent orofacial edema, recurrent facial nerve palsy, and lingua plicata. We retrospectively reviewed the medical records of 36 patients (24 women and 12 men) with elements of the Melkersson-Rosenthal syndrome. The complete triad was present in 9 (25%) patients. Orofacial involvement was the dominant feature; it occurred in all 36 patients and was the presenting sign in 15 (42%). Lingua plicata occurred in 18 (50%) patients, and peripheral facial paralysis was present in 17 (47%). Fourteen biopsy specimens were obtained, all from the orofacial region. Eight specimens revealed the classic pathologic picture of granulomatous cheilitis. No etiologic agent was identified in any of the patients. Diagnosis is difficult when all features of the triad are not present. A conservative treatment approach is recommended.