Medical and Dental Consultants’ Association of Nigeria
Home - About us - Editorial board - Search - Ahead of print - Current issue - Archives - Submit article - Instructions - Subscribe - Advertise - Contacts - Login 
  Users Online: 3075   Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
 

  Table of Contents 
CASE REPORT
Year : 2011  |  Volume : 14  |  Issue : 4  |  Page : 477-478

Melkerssons-Rosenthal syndrome: A case report and review of the literature


Department of Medicine, State Hospital, Abeokuta, Ogun State, Nigeria

Date of Acceptance02-Feb-2011
Date of Web Publication12-Jan-2012

Correspondence Address:
O A Talabi
Department of Medicine, State Hospital, Abeokuta, Ogun State
Nigeria
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1119-3077.91760

Rights and Permissions
   Abstract 

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


How to cite this article:
Talabi O A. Melkerssons-Rosenthal syndrome: A case report and review of the literature. Niger J Clin Pract 2011;14:477-8

How to cite this URL:
Talabi O A. Melkerssons-Rosenthal syndrome: A case report and review of the literature. Niger J Clin Pract [serial online] 2011 [cited 2019 Oct 21];14:477-8. Available from: http://www.njcponline.com/text.asp?2011/14/4/477/91760


   Introduction Top


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]


   Case Report Top


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

Click here to view
Figure 2: Placation of tongue

Click here to view


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.

 
   References Top

1.Balevi B. Melkersson-Rosenthal Syndrome: Review of the literature and case report of a 10 year misdiagnosis. Quintessence Int 1997;28:265-9.  Back to cited text no. 1
[PUBMED]    
2.Green RM, Rogers RS. Melkersson-Rosenthal Syndrome: A review of 36 patients'. J Am Acad Dermatol 1989;21;1263-70.  Back to cited text no. 2
    
3.Glickman LT, Gruss JS, Birt BD, Kohli-Dang N. The surgical management of Melkersson-Rosenthal Syndrome. Plast Reconstr Surg 1992;89:815-21.  Back to cited text no. 3
[PUBMED]    
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.  Back to cited text no. 4
    
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.  Back to cited text no. 5
    
6.Marques C, Machado A, Baptista AP. Marcrochelititis and Melkers son- Rosenthal syndrome. Review of 19 cases. Acta Med Port 1994;7:533-40.  Back to cited text no. 6
[PUBMED]    
7.Hintjens J, Daems L, Boss uyt M. Melkersson Rosenthal syndrome. Review of the literature. Acta Stomatol Belg 1994;91:143 -54.   Back to cited text no. 7
    
8.Cousin F, Grezard P, Berard F, Perrot H. Melkersson-Rosenthal syndrome. Ann Med interne (Paris) 1998;149:495-501.  Back to cited text no. 8
[PUBMED]    
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.   Back to cited text no. 9
    
10.Melkersson-Rosenthal syndrome: A case report. Ann Allergy Asthma Immunol 2002;88:421-4.  Back to cited text no. 10
    
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.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]


This article has been cited by
1 Melkersson–Rosenthal syndrome
J M Placke,M Moelleken,J Dissemond
QJM: An International Journal of Medicine. 2018; 111(3): 199
[Pubmed] | [DOI]
2 Melkersson-Rosenthal Syndrome with Orofacial Swelling and Recurrent Lower Motor Neuron Facial Nerve Palsy: A Case Report and Review of the Literature
Jerome Okudo,Yemi Oluyide
Case Reports in Otolaryngology. 2015; 2015: 1
[Pubmed] | [DOI]



 

Top
  
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case Report
    References
    Article Figures

 Article Access Statistics
    Viewed3885    
    Printed123    
    Emailed2    
    PDF Downloaded1126    
    Comments [Add]    
    Cited by others 2    

Recommend this journal