Melkerssons-Rosenthal syndrome: A case report and review of the literature
OA Talabi
Department of Medicine, State Hospital, Abeokuta, Ogun State, Nigeria
Date of Acceptance 02-Feb-2011
O A Talabi
Department of Medicine, State Hospital, Abeokuta, Ogun State
Melkerssons-Rosenthal syndrome is a clinical entity identified by the presence of the triad of recurrent facial paralysis, recurrent often permanent (labial) oedema, and to a lesser extent the placation of the tongue. The striking event is that of recurrent lower motor facial paresis. This should arouse the search for the other components of the triad which is not always complete. The major worry of the patients is however the facial paresis/oedema and the attendant inability to close the eyes which might sometimes get infected. Hallmark of management remains physical therapy and corticosteroid and/or antiviral agents. A high index of suspicion is required to make the diagnosis of Melkerssons-Rosenthal syndrome.
Keywords: Melkerssons-Rosenthal, Syndrome, Triad, Index of Suspicion
Talabi O A. Melkerssons-Rosenthal syndrome: A case report and review of the literature. Niger J Clin Pract 2011;14:477-8
Talabi O A. Melkerssons-Rosenthal syndrome: A case report and review of the literature. Niger J Clin Pract [serial online] 2011 [cited 2020 Jan 19];14:477-8. Available from: http://www.njcponline.com/text.asp?2011/14/4/477/91760
Melkerssons-Rosenthal syndrome is an uncommon neurocutneous disorder which often present with recurrent episodes of orofacial swelling, lower motor facial palsy, and fissured tongue. It has also been regarded as a non-caseating granulomatous disease of unclear etiology. [1],[2],[3],[5]
Presentations may be partial or complete. Many at times the main feature at presentation will be just facial edema involving the eyelids. [4],[5] Etiology and pathogenesis of MRS have not been fully determined. Several factors, such as infection, autoimmunity, neurotropic factors, atopy, and hypersensitivity to food additives have been implicated in the pathogenesis, but none of them are clearly proven. [6],[7],[8],[9]
Melkerssons-Rosenthal syndrome is defined by the triad of recurrent facial paralysis, recurrent often permanent (labial) oedema, and rarely placation of the tongue.
This 35-year-old woman whose consent was sorted, presented with four episodes of recurrent right lower motor facioparesis, right faciolabial oedema, with placation noticed over her tongue [Figure 1] and [Figure 2].
Figure 1: Melkerssons-Rosenthal syndrome
Figure 2: Placation of tongue
On the first two occasions she was treated with oral prednisolone and physical therapy with significant clinical improvement with each treatment only to recur.
On presentation, at the last and forth occasion she was treated with acyclovir 400 mg five times daily for 10 days in addition to prednisolone 60 mg daily for 6 days which was subsequently tapered off over the next 6 days.
She has since remained asymptomatic with no recurrence in the last 24 months. We still keep a monthly contact with her.
Ethical approval for this case report was obtained from the ethical committee of the State Hospital, Abeokuta, Nigeria. Patients' informed consent of the use of her picture for this article was also obtained.
Another possible mechanism is the vasomotor disturbances of both the vasa nervorum and the small arterioles of the subcutaneous tissues in response to unspecified stimuli in predisposed persons. An autosomal dominant inheritance with variable expression has been proposed in some cases of MRS
If the etiology is unclear, the diagnosis and course of treatment are not well defined. [9]
Various modalities of treatment have been tried, among which include corticosteroid, doxycycline, clofazimine, and thalidomide among others. [10],[11] A familial tendency has been reported. [11]
The presented case, after three clear episodes of about 2-3 months intervals has remained quiet after the forth for over 2 years now.
1. Balevi B. Melkersson-Rosenthal Syndrome: Review of the literature and case report of a 10 year misdiagnosis. Quintessence Int 1997;28:265-9.
2. Green RM, Rogers RS. Melkersson-Rosenthal Syndrome: A review of 36 patients'. J Am Acad Dermatol 1989;21;1263-70.
3. Glickman LT, Gruss JS, Birt BD, Kohli-Dang N. The surgical management of Melkersson-Rosenthal Syndrome. Plast Reconstr Surg 1992;89:815-21.
4. Ghorbel IB, SioudDhrif A, Lamioum, Trabelsi S, Habib Houman M. Melkersson-Rosenthal syndrome. Report of five cases and review of the Literature. Tunis Med 2006;84:816-20.
5. Marques C, Machado A, Baptista AP. Marcrochelitis and Melkersson-Rosenthal Syndrome. Report of five cases and review of the literature Tunis Med, 2006 Dec; 84:816-20.
6. Marques C, Machado A, Baptista AP. Marcrochelititis and Melkers son- Rosenthal syndrome. Review of 19 cases. Acta Med Port 1994;7:533-40.
7. Hintjens J, Daems L, Boss uyt M. Melkersson Rosenthal syndrome. Review of the literature. Acta Stomatol Belg 1994;91:143 -54.
8. Cousin F, Grezard P, Berard F, Perrot H. Melkersson-Rosenthal syndrome. Ann Med interne (Paris) 1998;149:495-501.
9. Medeiros M Jr, Araujo MI, Guimarães NS, Freitas LA, Silva TM, Carvalho EM. Therapeutic response to thalidomide in Melkersson-Rosenthal syndrome: A case report. Ann Allergy Asthma Immunol 2002;88:421-4.
10. Melkersson-Rosenthal syndrome: A case report. Ann Allergy Asthma Immunol 2002;88:421-4.
11. Safa G, Joly P, Boullie MC, Thomine E, Lauret P. Melkers son-Ros enthal syndrome: Treated by Thalidomide: 2 Cases. Am Dermatol Venereol 1995:122:609-11.
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Talabi O A
Melkerssons-Rosenthal
Index of Suspicion
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