eng Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2021-03-30 9 6 342 344 10.12691/ajmcr-9-6-10 AJMCR20219610 article Salem Bouomrani salembouomrani@yahoo.fr 1 2 Abir Masmoudi 2 2 Sihem Riahi 2 2 Mohamed Amin Bouaziz 2 2 Nour Wali 2 2 Warda Mahdhaoui 2 2 Department of Internal medicine, Military Hospital of Gabes, Gabes 6000, Tunisia 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. http://pubs.sciepub.com/ajmcr/9/6/10/ajmcr-9-6-10.pdf Melkersson-Rosenthal syndrome Beh?et disease systemic vasulitis granulomatosis lingua plicata