Melkersson-Rosenthal Syndrome Associated to Behçet Disease

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

¹Department of Internal medicine, Military Hospital of Gabes, Gabes 6000, Tunisia

²Sfax Faculty of Medicine, University of Sfax, Sfax 3029, Tunisia

Cite this paper

Salem Bouomrani, Abir Masmoudi, Sihem Riahi, Mohamed Amin Bouaziz, Nour Wali and Warda Mahdhaoui. Melkersson-Rosenthal Syndrome Associated to Behçet Disease. American Journal of Medical Case Reports. 2021; 9(6):342-344. doi: 10.12691/AJMCR-9-6-10

Abstract

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.

Keywords

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

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

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