Salem Bouomrani^{1, 2,}, Abir Masmoudi^{1, 2}, Sihem Riahi^{1, 2}, Mohamed Amin Bouaziz^{1, 2}, Nour Wali^{1, 2}, Warda Mahdhaoui^{1, 2}

Melkersson-Rosenthal syndrome (MRS) is an extremely rare disorder whose complete and typical form associates the triad: recurrent nonpitting orofacial edema, facial paralysis, and lingua plicata (fissured dorsal tongue). The exact pathogenesis of this disease is not yet fully understood. Its association with several other autoimmune disorders and the granulomatous nature of this syndrome suggest a dysimmune origin. The association with systemic vasculitis remains unusual with only two cases previously reported. We report the original case of MRS associated with Behçet disease in a 36-year-old Tunisian woman with favorable outcome under colchicine. Our observation is, to our knowledge, the first to report this association.

Melkersson-Rosenthal syndrome, Behçet disease, systemic vasulitis, granulomatosis, lingua plicata