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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.

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Furrowed tongue

MedGen UID:: 21583
•Concept ID:: C0040412
•: Anatomical Abnormality

Accentuation of the grooves on the dorsal surface of the tongue.

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Facial edema

MedGen UID:: 154241
•Concept ID:: C0542571
•: Pathologic Function

Swelling due to an excessive accumulation of fluid in facial tissues.

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Abnormality of the eye

MedGen UID:: 1370071
•Concept ID:: C4316870
•: Anatomical Abnormality

Any abnormality of the eye, including location, spacing, and intraocular abnormalities.

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Abnormality of head or neck
- Facial edema
- Furrowed tongue
Abnormality of the eye
- Abnormality of the eye
Abnormality of the musculoskeletal system
- Facial palsy

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMelkersson-Rosenthal syndrome

Pathological process
- Disease
  - Disorder by Site
    - Disorder of nervous system
      - Cranial nerve neuropathy
        Facial nerve disorder
        Melkersson-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

What is the most effective treatment of cheilitis granulomatosa in Melkersson-Rosenthal syndrome?

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

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

See all (6)

Recent clinical studies

Etiology

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

A Surprising Granulomatous Cheilitis.

Charpentier C, Kottler D, Fite C, Pelletier AL, Deschamps L, Descamps V
Gastroenterology 2018 Apr;154(5):1239-1240. Epub 2017 Jul 13 doi: 10.1053/j.gastro.2017.07.005. PMID: 28712755

Granulomatous diseases: Kids are not just little people.

Lakdawala N, Ferenczi K, Grant-Kels JM
Clin Dermatol 2017 Nov-Dec;35(6):555-565. Epub 2017 Aug 4 doi: 10.1016/j.clindermatol.2017.08.006. PMID: 29191347

Outcomes of recurrent facial palsy in Melkersson Rosenthal syndrome.

Wang J, Li P, Jin X, Xu Y, Zhang X
Ann Otol Rhinol Laryngol 2015 Mar;124(3):232-4. Epub 2014 Nov 10 doi: 10.1177/0003489414551981. PMID: 25385841

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

See all (69)

Diagnosis

Persistent Lip Swelling.

Kim S, Waterman CL, Hoyer S, Braniecki M
Cutis 2022 May;109(5):E32-E34. doi: 10.12788/cutis.0533. PMID: 35856748

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: 34137235 Free PMC Article

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

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Therapy

Current treatment modalities in granulomatous cheilitis.

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

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.

Ang KL, Jones NS
J Laryngol Otol 2002 May;116(5):386-8. doi: 10.1258/0022215021910861. PMID: 12081001

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

Danazol.

Madanes AE, Farber M
Ann Intern Med 1982 May;96(5):625-30. doi: 10.7326/0003-4819-96-5-625. PMID: 7041729

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Prognosis

Current treatment modalities in granulomatous cheilitis.

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

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

Granulomatous diseases: Kids are not just little people.

Lakdawala N, Ferenczi K, Grant-Kels JM
Clin Dermatol 2017 Nov-Dec;35(6):555-565. Epub 2017 Aug 4 doi: 10.1016/j.clindermatol.2017.08.006. PMID: 29191347

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

See all (66)

Clinical prediction guides

Current treatment modalities in granulomatous cheilitis.

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

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: 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

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

Observations on Melkersson's syndrome.

Scott GA
Postgrad Med J 1968 Jun;44(512):447-52. doi: 10.1136/pgmj.44.512.447. PMID: 5665744 Free PMC Article

See all (37)