Melkersson Rosenthal SendromuKlinik ve Etiyolojik Özellikleri: 3 Olgu Sunumu

Melkersson-Rosenthal sendromu (MRS); tekrarlayan periferik fasiyal paralizi, asimetrik fasiyal ödem ve fi ssürlü dil (lingua plicata) ile karakterize nadir görülen bir hastalıktır. Bu triad hastaların %25’inde görülür. Etiyoloji net olmamakla birlikte otoimmün lenfositik tablo ön plandadır. 20-40 yaş arasında 3 hasta kadın hasta, tekrarlayan fasiyal paralizi yakınması ile başvurdu. Fissürlü dil ve orofasiyal ödem saptandı. Ailede benzer bulgular yoktu. Vaskülitik, enfektif, metabolik, granülomatoz, paraneoplastik ve santral sinir sistemi hastalıklarına ait tüm tetkikler normaldi. Oral kortikosteroid tedavi ile şikâyetlerde azalma oldu. Hastalarının hepsinin ilkbahar aylarında periferik fasiyal paralizi atakları geçirmesi dikkati çekti. Bu durum alerjik etkenlerin etiyoloji ile ilişkilendirilebileceğini düşündürmüştür. MRS’nun insidansı % 0,08’dir. Olgular genellikle monosemptomatiktir. Otozomal dominant geçebilse de kanıtlar yetersizdir( Sakarya Tıp Dergisi, 2018, 8(4):863-868)

Clinical and Etiological Characteristics of Melkersson Rosenthal Syndrome: 3 Case Reports

Melkersson-Rosenthal syndrome (MRS) is a rare disorder characterized with recurrent peripheral facial paralysis, asymmetrical facial edemaand lingua plicata. 25% of patients have this triad. Etiology is unclear, but autoimmune lymphocytic condition may responsible. Threepatients, 20-40 years old were admitted with recurrent facial paralysis. Lingua plicata and orofacial edema were detected. There were nosimilar fi ndings in the family. All tests of vasculitic, infective, metabolic, granulomatosis, paraneoplastic and central nervous system diseaseswere normal. Complaints decreased with oral corticosteroid treatment. It was interesting that all patients had recurrent of peripheral facialparalysis in the spring. This suggested that allergens may be associated with etiology. The incidence of MRS is 0.08%. The cases are usuallymonosymptomatic. Despite MRS can be autosomal dominant, genetic evidence is not clear. ( Sakarya Med J, 2018, 8(4):863-868 ).

PDF

___

1. Ziem PE, Pfrommer C, Goerdt S, Orfanos CE, Blume Peytavi U. Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol. 2000;143:860-863.
2. Micheal S, Sara P, Henry S. Melkersson-Rosenthal syndrome in the perioculer area: A review of the literature and case report. Ann Plastic Surg 2003;150:664-668.
3. Apaydin R, Bilen N, Bayramgurler D, Efendi H, Vahaboglu H. Detection of Mycobacterium tuberculosis DNA in a patient with MelkerssonRosenthal syndrome using polymerase chain reaction. Br J Dermatol. 2000;142:1251-1252.
4. Kesler A, Vainstein G, Gadot N. Melkersson-Rosenthal syndrome treated by methylprednisolone. Neurology 1998;51:1440-1441.
5. Kruse-Losler B, Presser D, Metze D, Joos U. Surgical tre¬atment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granuloma¬tosa. Arch Dermatol. 2005;141:1085-1091.
6. Akdağ M, Özkurt FE, Yılmaz B, Topçu İ, Meriç F. Bilateral ve tekrarlayan fasiyal paralizinin nadir nedeni: Melkersson-Rosenthal sendromu. Dicle Tıp Dergisi 2015;42:102-106.
7. Khandpur S, Malhotra AK, Khanna N. Melkersson-Rosenthal syndrome with diffuse facial swelling and multiple cranial nerve palsies. J Dermatol. 2006;33:411-414.
8. Sciubba JJ, Said-Al-Naief N. Orofacial granulomatosis: presentation, pathology and management of 13 cases. J Oral Pathol Med. 2003;32:576- 585.
9. Cockerham KP, Hidayat AA, Cockerham GC, Depper M, Sorensen S, Cytyrn AS, et al. Melkersson-Rosenthal syndrome: new clinicopathologic fi ndings in 4 cases. Arch Ophthalmol. 2000;118:227-232.
10. Jasinska D, Boczon J. Melkersson–Rosenthal syndrome as an early manifestation of mixed connective tissue disease. European journal of medical research 2015;20:100