Melkersson-Rosenthal syndrome

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Classification according to ICD-10
G51.2 Melkersson-Rosenthal syndrome
ICD-10 online (WHO version 2019)

The Melkersson-Rosenthal syndrome (MRS, synonym orofacial granulomatosis ) is a rare idiopathic inflammatory disease with the three cardinal symptoms fissured tongue ( lingua plicata ), granulomatous cheilitis and facial paralysis .

Lingua plicata with increased longitudinal and / or transverse wrinkling
Bell's paresis with paralysis of the right facial muscles (left in the picture)

The name refers to the Swede Ernst Melkersson (1898-1932) and the Breslau-born doctor Curt Rosenthal (1892-1937).

The syndrome is not to be confused with Rosenthal-Klöpfer syndrome .

Spread and cause

The incidence is estimated at 8 in 10,000, the cause is not known, some hereditary predisposition ( autosomal dominant ) is suspected. Mutations detected so far were localized on chromosome 9 locus p11.

Autosomal dominant inheritance in Melkersson-Rosenthal syndrome

Clinical manifestations

Clinical criteria are:

  • Onset of illness in childhood or early adolescence
  • intensely furrowed tongue ( lingua plicata )
  • Sudden swelling in the area of ​​the lips due to enlarged lip salivary glands (as cheilitis granulomatosa ) or of the face (area of ​​the eyes, cheeks, forehead or palate) with granulomatous inflammation ( arteritis ).
  • Attack-like peripheral facial paralysis only occurs in 10% of cases (caused by swelling of the facial nerve in the canal n. VII). However, paresis does not occur at all or occurs much later. A triad is only found in rare cases. Usually it is a monosymptomatic course (as granulomatous cheilitis ).

Differential diagnosis

To be distinguished are recurrent erysipelas , herpes simplex infections, furuncles of the upper lip , macrocheilia and angioedema , including Ascher's syndrome .

course

In general , there is spontaneous remission , but there is a tendency to relapse . There is a frequent association with Crohn's disease and occasionally with sarcoid .

therapy

The treatment consists of an intralesional glucocorticoid injection (in the case of severe swelling) and the administration of clofazimin (Thesen 2005) or dapsone (both actually leprosy drugs). There are individual reports of healing successes with thalidomide .

In the case of slight swelling, cortisone helps e.g. B. Prednisolone (1 mg / KG body weight) for 2 weeks.

An operation can possibly be considered to correct cosmetically or mechanically disruptive conditions.

literature

  • R. Franz, C. Andres: Granulomatous cheilitis and Melkersson-Rosenthal syndrome. Intralymphatic histiocytosis as a valuable diagnostic indicator. In: The Pathologist. Volume 35, Number 2, March 2014, pp. 177-181, doi: 10.1007 / s00292-013-1850-6 , PMID 24619529 .
  • F. Nagel, R. Foelster-Holst: Granulomatous cheilitis. Melkersson-Rosenthal syndrome. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Volume 57, Number 2, February 2006, pp. 121-126, doi: 10.1007 / s00105-005-0981-y , PMID 15977013 (review).
  • Christoph Zink, Willibald Pschyrembel , Otto Dornblüth : Pschyrembel Clinical Dictionary. 255th edition. Walter de Gruyter, 1986, ISBN 3-11-007916-X , p. 1044 f.
  • Gerd Plewig, Michael Landthaler, Walter. HC Burgdorf, Michael Hertl, Thomas Ruzicka: Braun Falco's Dermatology, Venereology and Allergology , 6th edition. Springer, 2012, ISBN 978-3-642-24162-8 , pp. 1310 f.

Individual evidence

  1. ↑ Entry on Melkersson-Rosenthal syndrome in Flexikon , a Wiki of the DocCheck company
  2. ↑ a b c Melkersson-Rosenthal syndrome. In: Orphanet (Rare Disease Database).
  3. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  4. ↑ a b c Gerd Plewig, Michael Landthaler, Walter. HC Burgdorf, Michael Hertl, Thomas Ruzicka (eds.): Braun Falco's dermatology, venereology and allergology . 6th edition. Springer, Berlin / Heidelberg 2012, ISBN 978-3-642-24162-8 , pp. 1310 .
  5. ^ E. Melkersson: Ett fall av recidiverande facialispares i samband med ett angioneurotiskt ødem. In: Hygiea , Stockholm, 1928, Vol., Pp. 90737-41
  6. ^ C Rosenthal: Clinical-hereditary contribution to constitutional pathology. In: Journal for the whole of neurology and psychiatry. 131, 1931, p. 475, doi: 10.1007 / bf02865984 .
  7. ↑ Werner Gottwald: Breslauer Doctors and their meaning for the medical terminology. In: Würzburg medical history reports. Volume 8, 1990, pp. 289-294; here: pp. 289 and 293
  8. ↑ Who named it
  9. ^ PE Ziem, C. Pfrommer, S. Goerdt, CE Orfanos, U. Blume-Peytavi: Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. In: The British journal of dermatology. Volume 143, Number 4, October 2000, pp. 860-863, PMID 11069473 (review).
  10. ↑ S. Feng, J. Yin, J. Li, Z. Song, G. Zhao: Melkersson-Rosenthal syndrome: a retrospective study of 44 patients. In: Acta oto-laryngologica. Volume 134, number 9, September 2014, pp. 977-981, doi: 10.3109 / 00016489.2014.927587 , PMID 24963969 .
  11. ^ Melkersson-Rosenthal syndrome.  In: Online Mendelian Inheritance in Man . (English)
  12. ↑ a b c Encyclopedia Dermatology
  13. ↑ a b Gerd Plewig, Michael Landthaler, Walter. HC Burgdorf, Michael Hertl, Thomas Ruzicka (eds.): Braun Falco's dermatology, venereology and allergology . 6th edition. Springer, Berlin / Heidelberg 2012, ISBN 978-3-642-24162-8 , pp. 1311 .
  14. ^ Pharmaceutical newspaper
  15. ^ Technical information from the Riemser company dated January 19, 2011
  16. ↑ F. Ziemssen, JM Rohrbach, C. Scherwitz, PO Denk: Plastic reconstructive correction of a persistent lid edema in Melkersson-Rosenthal syndrome. In: Clinical monthly sheets for ophthalmology. Volume 220, Number 5, May 2003, pp. 352-356, doi: 10.1055 / s-2003-39431 , PMID 12766825 .