Macrocheilitis and Melkersson-Rosenthal syndrome. Review of 19 cases.

Abstract

Melkersson-Rosenthal syndrome is an uncommon condition of unknown cause. The classical triad includes recurrent orofacial oedema involving predominantly the lips (macrocheilitis), intermittent peripheral facial palsy and scrotal tongue. Some authors consider Miescher's cheilitis as a monosymptomatic form of the syndrome. Nineteen cases (11 females and 8 males) of macrocheilitis seen at the Dermatologic Clinic, University of Coimbra, between 1966 and 1992, are reviewed. The complete triad of signs was present only in 4 patients (21%) and two features of the classic triad in 3 (16%) (macrocheilitis and lingua plicata in two; macrocheilitis and facial palsy in one). Twelve patients had only recorrent or persistent orofacial swelling. All 19 patients had macrocheilitis. Less commonly, the check, nose, forehead, eyelid, chin and buccal mucosa were also involved. Peripheral facial paralysis was present in 5 cases (26%), and scrotal tongue occurred in 6 (32%). Lip biopsies, performed in 17 patients, revealed the typical pathologic picture of granulomatous cheilitis only in 8 cases. Systemic corticosteroids and clofazimine were the most efficacious treatment.