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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.
Furrowed tongue
MedGen UID:
21583
Concept ID:
C0040412
Anatomical Abnormality
Accentuation of the grooves on the dorsal surface of the tongue.
Facial edema
MedGen UID:
154241
Concept ID:
C0542571
Pathologic Function
Swelling due to an excessive accumulation of fluid in facial tissues.
Abnormality of the eye
MedGen UID:
1370071
Concept ID:
C4316870
Anatomical Abnormality
Any abnormality of the eye, including location, spacing, and intraocular abnormalities.

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

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
Sobjanek M, Michajłowski I, Zelazny I, Medrzycka-Dabrowska W, Włodarkiewicz A
J Eur Acad Dermatol Venereol 2010 Mar;24(3):364-5. Epub 2010 Jan 11 doi: 10.1111/j.1468-3083.2009.03540.x. PMID: 20070456
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

Gavioli CFB, Nico MMS, Florezi GP, Lourenço SV
J Cutan Pathol 2020 Nov;47(11):1010-1017. Epub 2020 Aug 6 doi: 10.1111/cup.13784. PMID: 32578236
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
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
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
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

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
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
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
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
Daoud MS, Rogers RS 3rd
Semin Dermatol 1995 Jun;14(2):135-9. doi: 10.1016/s1085-5629(05)80009-4. PMID: 7640193

Therapy

Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
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
Rose AE, Leger M, Chu J, Meehan S
Dermatol Online J 2011 Oct 15;17(10):15. PMID: 22031641
Ang KL, Jones NS
J Laryngol Otol 2002 May;116(5):386-8. doi: 10.1258/0022215021910861. PMID: 12081001
Madanes AE, Farber M
Ann Intern Med 1982 May;96(5):625-30. doi: 10.7326/0003-4819-96-5-625. PMID: 7041729

Prognosis

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
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
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
Daoud MS, Rogers RS 3rd
Semin Dermatol 1995 Jun;14(2):135-9. doi: 10.1016/s1085-5629(05)80009-4. PMID: 7640193
Vistnes LM, Kernahan DA
Plast Reconstr Surg 1971 Aug;48(2):126-32. PMID: 4934902

Clinical prediction guides

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
Pathania YS
Int J Dermatol 2022 Jun;61(6):755-759. Epub 2022 Feb 17 doi: 10.1111/ijd.16126. PMID: 35174873
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
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
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

Recent systematic reviews

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
Wehl G, Rauchenzauner M
Curr Pediatr Rev 2018;14(3):196-203. doi: 10.2174/1573396314666180515113941. PMID: 29766816
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
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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