MELKERSSON-ROSENTAL SYNDROME IN CHILDREN AS AN ORPHAN DISEASE: PREVALENCE, CLINICS AND TREATMENT METHODS

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Abstract

In this article, the authors, referring to their own practice, cases from practice, present information about a rare syndrome in children - Melkersson-Rosenthal. Based on the results of their own clinical observation, instrumental and laboratory studies, the clinical and diagnostic criteria of the syndrome are described. Historical and modern literary data on the rare Melkersson-Rosenthal syndrome are provided. The authors argue that the disease begins during the transition from childhood to adolescence, and after treatment, the progression of the disease slows down, but does not stop, which requires close monitoring by both pediatricians and general practitioners.

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List of references

Zhukova O.V., Atabieva A.Ya., Tereshchenko A.V., Dolya O.V., Utkin D.V. Macrocheilitis as a manifestation of Melkersson-Rosenthal syndrome. Medical Council. 2022;16(23):299–303. https://doi.org/10.21518/2079-701X-2022-16-23-299-303. DOI: https://doi.org/10.21518/2079-701X-2022-16-23-299-303

Bruett C.T., Trump B.R., Adams D.R., Halpern L.R. Orofacial granulomatosis: A case treated with azithromycin pulse therapy, review of the literature and an algorithm for diagnosis. ID Cases. 2020;e00924. https://doi.org/10.1016/j.idcr.2020.e00924. DOI: https://doi.org/10.1016/j.idcr.2020.e00924

Değerli Ş., Başman A., Gumuşok M., Kaya M., Alkurt M.T. Melkersson – Rosenthal syndrome: a case report. J Istanb Univ Fac Dent. 2017;51(1):42–45. https://doi.org/10.17096/jiufd.96279. DOI: https://doi.org/10.17096/jiufd.96279

Dhawan S.R., Saini A.G., Singhi P.D. Management Strategies of Melkersson – Rosenthal Syndrome: A Review. Int J Gen Med. 2020;13:61–65. https://doi.org/10.2147/IJGM.S186315. DOI: https://doi.org/10.2147/IJGM.S186315

Emiroglu N., Su O., Cengiz F.P., Onsun N. Successful treatment of Melkersson-Rosenthal syndrome with dapsone: a case report and review of the literature. Dermatol Online J. 2016;22(10):13030/qt4zz1g8t1. https://doi.org/10.5070/D32210032911. DOI: https://doi.org/10.5070/D32210032911

Fedele S., Fung P., 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.12655. DOI: https://doi.org/10.1111/bjd.12655

Gaudio A., Corrado A., Santoro N., Maruotti N., Cantatore F.P. Melkersson-rosenthal syndrome in a patient with psoriatic arthritis receiving etanercept. Int J Immunopathol Pharmacol. 2013;26(1):229–233. https://doi.org /10.1177/039463201302600123. DOI: https://doi.org/10.1177/039463201302600123

James, William D.; Berger, Timothy G.; et al. Andrews' Diseases of the Skin: Clinical Dermatology (англ.). – Saunders Elsevier, 2006. — ISBN 0-7216-2921-0.

Medeiros M.Jr., Araujo M.I., Guimaraes N.S., Freitas L.A., Silva T.M., Carvalho E.M. Therapeutic response to thalidomide in Melkersson – Rosenthal syndrome: a case report. Ann Allergy Asthma Immunol. 2002;88(4):421–424. https://doi.org/10.1016/S1081-1206(10)62375-7. DOI: https://doi.org/10.1016/S1081-1206(10)62375-7

Melkersson E. Ett fall av recideverande facial spares. Samband Med Angioneurotisk Odem Hygeia (Stockholm). 1928;90:737–741.

Orlando M.R., Atkins J.S. Melkersson – Rosenthal syndrome. Arch Otolaryngol Head Neck Surg. 1990;116(6):728–729. https://doi.org/10.1001/archotol.1990.01870060086017. DOI: https://doi.org/10.1001/archotol.1990.01870060086017

Pachor M.L., Urbani G., Cortina P., Lunardi C., Nicolis F., Peroli P. et al. Is the Melkersson – Rosenthal syndrome related to the exposure to food additives? A case report. Oral Surg Oral Med Oral Pathol. 1989;67(4):393–395. https://doi.org/10.1016/0030-4220(89)90380-0. DOI: https://doi.org/10.1016/0030-4220(89)90380-0

Rosenthal C. Gemeinsames Auftreten von (rezidivierender familiarer) Facialislahmung, angioneurotischem Gesichtsodem und Lingua plicatain Arthritismus-Familien. Zeitschrift fur die gesamte Neurologie und Psychiatrie. 1931;131:475. DOI: https://doi.org/10.1007/BF02865984

Saini A.G., Sankhyan N., Padmanabh H., Das A., Singhi P. Recurrent facial palsy and electrophysiological findings in oligosymptomatic Melkersson Rosenthal syndrome. Indian J Pediatr. 2016;83(10):1188–1190. https://doi.org/10.1007/s12098-016-2137-8. DOI: https://doi.org/10.1007/s12098-016-2137-8

Savasta S., Rossi A., Foiadelli T., Licari A., Elena Perini A.M., 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):1289. https://doi.org/10.3390/ijerph16071289. DOI: https://doi.org/10.3390/ijerph16071289

Stein J., Paulke A., Schacher B., Noehte M. An extraordinary form of the Melkersson-Rosenthal syndrome successfully treated with the tumour necrosis factor-alpha blocker adalimumab. BMJ Сase Rep. 2014;2014:bcr2014204674. https://doi.org/10.1136/bcr-2014-204674. DOI: https://doi.org/10.1136/bcr-2014-204674

Tang J.-J., Shen X., Xiao J.-J., Wang X.-P. Retrospective analysis of 69 patients with Melkersson-Rosenthal syndrome in mainland China. Int J Clin Exp Med. 2016;9(2):3901–3908.

Tonkovic-Capin V., Galbraith S.S., Rogers R.S. 3rd, Binion D.G., Yancey K.B. Cutaneous Crohn’s disease mimicking Melkersson-Rosenthal syndrome: treatment with methotrexate. J Eur Acad Dermatol Venereol. 2006;20(4):449–452. https://doi.org/10.1111/j.1468-3083.2006.01458.x. DOI: https://doi.org/10.1111/j.1468-3083.2006.01458.x

Wehl G., Rauchenzauner M. A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson – Rosenthal Syndrome. Curr Pediatr Rev. 2018;14(3):196–203. https://doi.org/10.2174/1573396314666180515113941. DOI: https://doi.org/10.2174/1573396314666180515113941

Wickramasinghe N., Gunasekara C.N., Fernando W.S., Hewavisenthi J., de Silva H.J. Vulvitis granulomatosa, Melkersson-Rosenthal syndrome, and Crohn’s disease: dramatic response to infliximab therapy. Int J Dermatol. 2012;51(8):966–968. https://doi.org/10.1111/j.1365-4632.2011.05177.x. DOI: https://doi.org/10.1111/j.1365-4632.2011.05177.x

How to Cite

Khamidov Ф., Botirov К., & Khamidov А. (2025). MELKERSSON-ROSENTAL SYNDROME IN CHILDREN AS AN ORPHAN DISEASE: PREVALENCE, CLINICS AND TREATMENT METHODS. International Journal of Scientific Pediatrics, 4(5), 1133–1137. https://doi.org/10.56121/2181-2926-2025-4-5-1133-1137
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