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Melkersson-Rosenthal syndrome(MRS)

MedGen UID:
6291
•Concept ID:
C0025235
•
Disease or Syndrome
Synonyms: Cheilitis Granulomatosa; Melkersson syndrome
SNOMED CT: Cheilitis granulomatosa of Miescher-Melkersson-Rosenthal (215617000); Macrocheilia, facial palsy AND edema syndrome (215617000); Melkersson-Rosenthal syndrome (37770007); Melkersson syndrome (37770007); Melkersson's syndrome (37770007)
 
Monarch Initiative: MONDO:0007969
OMIM®: 155900
Orphanet: ORPHA2483

Definition

Melkersson-Rosenthal syndrome is characterized by chronic swelling of the face, peripheral facial palsy, which may be bilateral and may tend to relapse, and in some cases ligua plicata (fissured tongue). The swelling is localized especially to the lips. Onset is usually in childhood or adolescence (summary by Kunstadter, 1965). [from OMIM]

Clinical features

From HPO
Facial palsy
MedGen UID:
87660
•Concept ID:
C0376175
•
Disease or Syndrome
Facial nerve palsy is a dysfunction of cranial nerve VII (the facial nerve) that results in inability to control facial muscles on the affected side with weakness of the muscles of facial expression and eye closure. This can either be present in unilateral or bilateral form.
See: Feature record | Search on this feature
Furrowed tongue
MedGen UID:
21583
•Concept ID:
C0040412
•
Anatomical Abnormality
Accentuation of the grooves on the dorsal surface of the tongue.
See: Feature record | Search on this feature
Facial edema
MedGen UID:
154241
•Concept ID:
C0542571
•
Pathologic Function
Swelling due to an excessive accumulation of fluid in facial tissues.
See: Feature record | Search on this feature
Abnormality of the eye
MedGen UID:
1370071
•Concept ID:
C4316870
•
Anatomical Abnormality
Any abnormality of the eye, including location, spacing, and intraocular abnormalities.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMelkersson-Rosenthal syndrome
Follow this link to review classifications for Melkersson-Rosenthal syndrome in Orphanet.

Professional guidelines

PubMed

Botulinum toxin in the treatment of facial synkinesis and hyperkinesis.
Filipo R, Spahiu I, Covelli E, Nicastri M, Bertoli GA
Laryngoscope 2012 Feb;122(2):266-70. Epub 2012 Jan 17 doi: 10.1002/lary.22404. PMID: 22252570
Clofazimine--an effective treatment for Melkersson-Rosenthal syndrome or Miescher's cheilitis.
Podmore P, Burrows D
Clin Exp Dermatol 1986 Mar;11(2):173-8. doi: 10.1111/j.1365-2230.1986.tb00443.x. PMID: 3720016
Facial paralysis in children: differential diagnosis.
May M, Fria TJ, Blumenthal F, Curtin H
Otolaryngol Head Neck Surg 1981 Sep-Oct;89(5):841-8. doi: 10.1177/019459988108900528. PMID: 6799919

Recent clinical studies

Etiology

Clinical Profile of Melkersson-Rosenthal Syndrome/Orofacial Granulomatosis: A Review of 51 Patients.
Gavioli CF, Florezi GP, Lourenço SV, Nico MM
J Cutan Med Surg 2021 Jul-Aug;25(4):390-396. Epub 2021 Feb 11 doi: 10.1177/1203475421995132. PMID: 33573395
Facial nerve decompression.
Andresen NS, Sun DQ, Hansen MR
Curr Opin Otolaryngol Head Neck Surg 2018 Oct;26(5):280-285. doi: 10.1097/MOO.0000000000000478. PMID: 30138146
Granulomatous & histiocytic dermatitides.
Wick MR
Semin Diagn Pathol 2017 May;34(3):301-311. Epub 2016 Dec 14 doi: 10.1053/j.semdp.2016.12.003. PMID: 28094163
Melkersson-Rosenthal syndrome: a review of seven patients.
Liu R, Yu S
J Clin Neurosci 2013 Jul;20(7):993-5. Epub 2013 May 7 doi: 10.1016/j.jocn.2012.10.009. PMID: 23664134
Melkersson-Rosenthal syndrome.
Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A
Otolaryngol Head Neck Surg 2008 Feb;138(2):246-51. doi: 10.1016/j.otohns.2007.11.015. PMID: 18241724

Diagnosis

Research progress on Melkersson-Rosenthal syndrome.
Kuang W, Luo X, Wang J, Zeng X
Zhejiang Da Xue Xue Bao Yi Xue Ban 2021 Apr 25;50(2):148-154. doi: 10.3724/zdxbyxb-2021-0103. PMID: 34137235Free PMC Article
A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson - Rosenthal Syndrome.
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
Orofacial granulomatosis.
Miest R, Bruce A, Rogers RS 3rd
Clin Dermatol 2016 Jul-Aug;34(4):505-13. Epub 2016 Mar 3 doi: 10.1016/j.clindermatol.2016.02.024. PMID: 27343966
Melkersson-Rosenthal syndrome.
Lin TY, Chiang CH, Cheng PS
J Formos Med Assoc 2016 Jul;115(7):583-4. Epub 2015 Sep 12 doi: 10.1016/j.jfma.2015.08.011. PMID: 26375775
Melkersson-Rosenthal syndrome.
Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A
Otolaryngol Head Neck Surg 2008 Feb;138(2):246-51. doi: 10.1016/j.otohns.2007.11.015. PMID: 18241724

Therapy

Melkersson-Rosenthal Syndrome Induced by COVID-19.
Taşlıdere B, Mehmetaj L, Özcan AB, Gülen B, Taşlıdere N
Am J Emerg Med 2021 Mar;41:262.e5-262.e7. Epub 2020 Aug 15 doi: 10.1016/j.ajem.2020.08.018. PMID: 32829989Free PMC Article
A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson - Rosenthal Syndrome.
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
Melkersson-Rosenthal syndrome.
Lin TY, Chiang CH, Cheng PS
J Formos Med Assoc 2016 Jul;115(7):583-4. Epub 2015 Sep 12 doi: 10.1016/j.jfma.2015.08.011. PMID: 26375775
Cheilitis granulomatosa.
Rose AE, Leger M, Chu J, Meehan S
Dermatol Online J 2011 Oct 15;17(10):15. PMID: 22031641
Melkersson-Rosenthal syndrome.
Ang KL, Jones NS
J Laryngol Otol 2002 May;116(5):386-8. doi: 10.1258/0022215021910861. PMID: 12081001

Prognosis

A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson - Rosenthal Syndrome.
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
Melkersson-Rosenthal syndrome.
Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A
Otolaryngol Head Neck Surg 2008 Feb;138(2):246-51. doi: 10.1016/j.otohns.2007.11.015. PMID: 18241724
Cheilitis granulomatosa.
van der Waal RI, Schulten EA, van de Scheur MR, Wauters IM, Starink TM, van der Waal I
J Eur Acad Dermatol Venereol 2001 Nov;15(6):519-23. doi: 10.1046/j.1468-3083.2001.00353.x. PMID: 11843210
Melkersson-Rosenthal syndrome.
Daoud MS, Rogers RS 3rd
Semin Dermatol 1995 Jun;14(2):135-9. doi: 10.1016/s1085-5629(05)80009-4. PMID: 7640193
The Melkersson-Rosenthal syndrome.
Vistnes LM, Kernahan DA
Plast Reconstr Surg 1971 Aug;48(2):126-32. PMID: 4934902

Clinical prediction guides

Surgical Treatment Outcome for Familial Melkersson-Rosenthal Syndrome.
Alencar TN, Botelho MM, Carasek N, Bahmad F Jr
Am J Case Rep 2023 Feb 9;24:e938670. doi: 10.12659/AJCR.938670. PMID: 36755481Free PMC Article
Clinical Profile of Melkersson-Rosenthal Syndrome/Orofacial Granulomatosis: A Review of 51 Patients.
Gavioli CF, Florezi GP, Lourenço SV, Nico MM
J Cutan Med Surg 2021 Jul-Aug;25(4):390-396. Epub 2021 Feb 11 doi: 10.1177/1203475421995132. PMID: 33573395
Melkersson-Rosenthal syndrome with coeliac and allergic diseases.
Martins JA, Azenha A, Almeida R, Pinheiro JP
BMJ Case Rep 2019 Aug 28;12(8) doi: 10.1136/bcr-2019-229857. PMID: 31466978Free PMC Article
Melkersson-Rosenthal syndrome: About a Tunisian family and review of the literature.
Mansour M, Mahmoud MB, A Kacem, Zaouali J, Mrissa R
Clin Neurol Neurosurg 2019 Oct;185:105457. Epub 2019 Aug 5 doi: 10.1016/j.clineuro.2019.105457. PMID: 31445326
Melkersson-Rosenthal syndrome.
Abebe M
Ethiop Med J 1989 Apr;27(2):85-8. PMID: 2714262

Recent systematic reviews

For Whom the Bell's Toll: Recurrent Facial Nerve Paralysis, A Retrospective Study and Systematic Review of the Literature.
Chweya CM, Anzalone CL, Driscoll CLW, Lane JI, Carlson ML
Otol Neurotol 2019 Apr;40(4):517-528. doi: 10.1097/MAO.0000000000002167. PMID: 30870370
A Systematic Review of the Literature of the Three Related Disease Entities Cheilitis Granulomatosa, Orofacial Granulomatosis and Melkersson - Rosenthal Syndrome.
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
Association between orofacial granulomatosis and Crohn's disease in children: systematic review.
Lazzerini M, Bramuzzo M, Ventura A
World J Gastroenterol 2014 Jun 21;20(23):7497-504. doi: 10.3748/wjg.v20.i23.7497. PMID: 24966621Free PMC Article
A comprehensive review of current treatments for granulomatous cheilitis.
Banks T, Gada S
Br J Dermatol 2012 May;166(5):934-7. doi: 10.1111/j.1365-2133.2011.10794.x. PMID: 22187977

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