Melkersson–Rosenthal syndrome. Clinical case
- Authors: Snarskaya E.S.1, Medvedeva Y.N.1
-
Affiliations:
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
- Issue: Vol 24, No 5 (2021)
- Pages: 461-466
- Section: CLINICAL PICTURE, DIAGNOSIS, AND THERAPY OF DERMATOSES
- URL: https://rjsvd.com/1560-9588/article/view/89544
- DOI: https://doi.org/10.17816/dv89544
- ID: 89544
Cite item
Abstract
This clinical case is of interest due to the rare occurrence of Melkersson–Rosenthal syndrome in the population, as well as the difficulty in diagnosing and achieving remission of this disease.
Melkersson–Rosenthal syndrome is a rare chronic relapsing dermatosis in the population, in typical cases characterized by a triad of symptoms: macrocheilitis, neuropathy of the facial nerve and folding of the tongue. However, much more often the disease manifests monosymptomically. The etiology of the syndrome has not been definitively studied: the infectious and allergic component, inflammatory processes of the facial nerve, genetic predisposition, as well as contact with metals (cobalt and gold) are considered as the determining causes. The diagnosis is based on clinical data and the results of a pathomorphological study. With a monosymptomatic (only the presence of macroheilitis) course of Melkersson–Rosenthal syndrome, a differential diagnosis is carried out with angioedema Quincke, lymphangioma, hemangioma, lip abscess and other cheilitis.
The clinical picture of dermatosis is localized on the face and is a significant problem, significantly affecting the social status of patients and is highly threatened in relation to the development of dysmorphophobia.
The presented clinical case is rare in the practice of a dermatologist, patients with a similar pathology turn to dentists, allergists, where the clinical picture is regarded as Quincke’s edema. Given the rare occurrence of Melkersson–Rosenthal syndrome in the population and the difficulty of diagnosing the disease, the presented clinical case is indicative, since in our case the patient has only 2 main symptoms (folded tongue, macrocheilitis) and there is no clinical picture of facial neuritis.
Timely diagnosis and a thorough examination of patients with Melkersson–Rosenthal syndrome contribute to the greatest effectiveness of therapy and the achievement of a stable remission of the disease.
Full Text
About the authors
Elena S. Snarskaya
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Author for correspondence.
Email: snarskaya-dok@mail.ru
ORCID iD: 0000-0002-7968-7663
SPIN-code: 3785-7859
MD, Dr. Sci. (Med.), Professor
Russian Federation, 2, building 2, Bolshaya Pirogovskaya st., Moscow, 119992Yana N. Medvedeva
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Email: dr.yana.med@bk.ru
ORCID iD: 0000-0003-2323-4339
MD
Russian Federation, MoscowReferences
- Esedov EM, Akhmetova FD. Diagnosis of Rossolimo-Melkersson-Roselthal syndrome. Bulletin of the DGMA. 2013;(4):13–17. (In Russ).
- Dorozhenok IY, Matyushenko EN, Olisova OY. Dysmorphophobia in dermatological practi. Russian Journal of Skin and Venereal Diseases. 2014;17(1):42–47. (In Russ).
- El-Hakim M, Chauvin P. Orofacial granulomatosis presenting as persistent lip swelling: review of 6 new cases. J Oral Maxillofac Surg. 2004;62(9):1114–1117. doi: 10.1016/j.joms.2003.11.013
- Xu XG, Guan LP, Lv Y, et al. Exome sequencing identifies FATP1 mutation in Melkersson-Rosenthal syndrome. J Eur Acad Dermatol Venereol. 2017;31(5):230–232. doi: 10.1111/jdv.14042
- Reznichenko NYu, Veretelnyk A.V. Case report of Melkersson-Rosenthal syndrome. Clinical Immunology. Allergology. Infectology. 2017;6(103):46–47. (In Ukrainian).
- Saalman R, Sundell S, Kullberg-Lindh C, et al. Long-standing oral mucosal lesions in solid organ-transplanted children-a novel clinical entity. Transplantation. 2010;89:606–611. doi: 10.1097/TP.0b013e3181ca7b04
- White A, Nunes C, Escudier M, et al. Improvement in orofacial granulomatosis on a cinnamon- and benzoate-free diet. Inflamm Bowel Dis. 2006;12(6):508–514. doi: 10.1097/00054725-200606000-00011
- Wong GA, Shear NH. Melkersson-Rosenthal syndrome associated with allergic contact dermatitis from octyl and dodecyl gallates. Contact Dermatitis. 2003;49(5):266–267. doi: 10.1111/j.0105-1873.2003.0225j.x
- Pochtar VN, Skiba VY, Skiba AV. Clinical case of Melkersson-Rosenthal syndrome. Clinical Dentistry. 2013;(2):26–30. (In Russ).
- Chen C, Huilgol SC, James C, Selva D. Melkersson-Rosenthal syndrome resenting with upper lid edema and facial palsy. Can J Ophthalmol. 2002;37(6):361–363. doi: 10.1016/s0008-4182(02)80008-4
- Chu Z, Liu Y, Zhang H, et al. Melkersson-Rosenthal syndrome with genitalia involved in a 12-year-old boy. Ann Dermatol. 2016;28(2):232–236. doi: 10.5021/ad.2016.28.2.232
- Feng S, Yin J, Li J, et al. Melkersson-Rosenthal syndrome: a retrospective study of 44 patients. Acta Otolaryngol. 2014;134(9):977–981. doi: 10.3109/00016489.2014.927587
- Brusenina ND, Rybalkina EA. Lip diseases: a textbook for the post-university system. prof. education of dentists. Ed. by G.M. Barer. Moscow; 2005. 184 p. (In Russ).