Resumen
El síndrome de Miescher-Melkerson-Rosenthal es una condición clínica poco frecuente caracterizada por un edema orofacial episódico, paresia facial periférica recurrente, lengua geográfica (lingua plicata) y granulomas en biopsia de piel o labial. Se ha reportado más frecuentemente en mujeres, y su incidencia es probablemente subestimada por el desconocimiento del síndrome, su presentación clínica incompleta y la plétora de diagnósticos diferenciales. Se debe considerar en pacientes que presenten al menos dos de las cuatro características mencionadas. Su tratamiento, con esteroides vía oral, está dirigido a los episodios de edema orofacial y/o paresia facial. A continuación se describen tres casos del síndrome valorados en el servicio de neurología de la Fundación Cardioinfantil y se presenta una breve revisión de la literatura.
Citas
Gavioli CFB, Nico MMS, Panajotopoulos N, Rodrigues H, Rosales CB, Valente NYS, et al. A case-control study of HLA alleles in Brazilian patients with Melkersson-Rosenthal syndrome. Eur J Med Genet. 2020;63(7):103879. https://doi.org/10.1016/j.ejmg.2020.103879
Pei Y, Beaman GM, Mansfield D, Clayton-Smith J, Stewart M, Newman WG. Clinical and genetic heterogeneity in Melkersson-Rosenthal syndrome. Eur J Med Genet. 2019;62(6):103536. https://doi.org/10.1016/j.ejmg.2018.09.003
Savasta S, Rossi A, Foiadelli T, Licari A, Perini AME, Farello G, et al. Melkersson-Rosenthal syndrome in childhood: Report of three paediatric cases and a review of the literature. Int J Environ Res Public Health. 2019;16(7):1-13. https://doi.org/10.3390/ijerph16071289.
Kajal B, Harvey J, Alowami S. Melkerrson-Rosenthal Syndrome, a rare case report of chronic eyelid swelling. Diagn Pathol. 2013;8:188. https://doi.org/10.1186/1746-1596-8-188
Martins JA, Azenha A, Almeida R, Pinheiro JP. Melkersson-Rosenthal syndrome with coeliac and allergic diseases. BMJ Case Rep. 2019;12(8):229857. https://doi.org/10.1136/bcr-2019-229857
Haaramo A, Kolho KL, Pitkaranta A, Kanerva M. A 30-year follow-up study of patients with Melkersson-Rosenthal syndrome shows an association to inflammatory bowel disease. Ann Med. 2019;51(2):149-55. https://doi.org/10.1080/07853890.2019.1591634
Apaydin R, Bahadir S, Kaklikkaya N, Bilen N, Bayramgurler D. Possible role of Mycobacterium tuberculosis complex in Melk-ersson-Rosenthal syndrome demonstrated with Gen-Probe amplified Mycobacterium tuberculosis direct test. Australas J Dermatol. 2004;45(2):94-9. https://doi.org/10.1111/j.1440-0960.2004.00071.x
Fantacci C, Mariotti P, Miceli Sopo S, Ferrara P, Rendeli C, Chiaretti A. Intravenous immunoglobulins in Melkersson-Rosenthal syndrome: A clinical and neuroimaging study. Pediatr Allergy Inmmunol. 2018;29(8):881-3. https://doi.org/10.1111/pai.12970
Cancian M, Giovannini S, Angelini A, Fedrigo M, Bendo R, Senter R, et al. Melkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features. Allergy Asthma Clin Immunol. 2019;15:1. https://doi.org/10.1186/s13223-018-0316-z
Lin TY, Chiang CH, Cheng PS. Melkersson-Rosenthal síndrome. J Formos Med Assoc. 2016;115(7):583-4. https://doi.org/10.1016/j.jfma.2015.08.011
Zeng W, Geng S, Niu X, Yuan J. Complete Melkersson-Rosen-thal syndrome with multiple cranial nerve palsies. Clin Exp Dermatol. 2010;25(3):272-4. https://doi.org/10.1111/j.1365-2230.2009.03418.x
Tang JJ, Shen X, Xiao JJ, Wang XP. Retrospective analysis of 69 patients with Melkersson-Rosenthal syndrome in mainland China. Int J Clin Exp Med. 2016;9(2):3901-8.
Mansour M, Mahmoud MB, Kacem A, Zaouali J, Mrissa R. Melkersson-Rosenthal syndrome: About a Tunisian family and review of the literature. Clin Neurol Neurosurg. 2019;185:105457. https://doi.org/10.1016/j.cli-neuro.2019.105457
Dhawan SR, Saini AG, Singhi PD. Management strategies of Melkersson-Rosenthal syndrome: A review. Int J Gen Med. 2020;13:61-5. https://doi.org/10.2147/IJGM.S186315
Fedele S, Fung PP, Bamashmous N, Petrie A, Porter S. Long-term effectiveness of intralesional triamcinolone acetonide therapy in orofacial granulomatosis: an observational cohort study. Br J Dermatol. 2014;170(4):794-801. https://doi.org/10.1111/bjd.2014.170.issue-4
Tan Z, Zhang Y, Chen W, Gong W, Zhao J, Xu X. Recurrent facial palsy in Melkersson Rosenthal syndrome: total facial nerve decompression is effective to prevent further recurrence. Am J Otolaryngol. 2015;36(3):334-7. https://doi.org/10.1016/j.amjoto.2014.12.001
Vilela DSA, Balieiro FO, Fernandes-Alessandro MF, Mitre EI, Lazarini PR. Síndrome de Melkersson-Rosenthal: relato de casos e revisão da literatura. Rev Bras Otorrinolaringol. 2002;68(5):755-60. https://doi.org/10.1590/S0034-72992002000500024
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