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Cheilitis granulomatosa

Melkersson-Rosenthal-Syndrom

Cheilitis granulomatosa

Melkersson-Rosenthal syndrome

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Zusammenfassung

Das Melkersson-Rosenthal-Syndrom (MRS) ist eine seltene Erkrankung, die durch die Symptomentrias von rezidivierender orofazialer Schwellung, rezidivierender Fazialisparese und Lingua plicata charakterisiert ist. Die komplette Trias tritt jedoch nur in etwa 25% aller MRS-Fälle auf. Häufiger sind mono- oder oligosymptomatische Formen. Zusätzlich gibt es noch andere Symptome, die mit dem MRS assoziiert auftreten können, wie Granulome in anderen Gesichtsregionen, regionale Lymphknotenvergrößerungen, Fieber, psychotische Störungen, Hyperplasie und Entzündungen der Gingiva. Die Ätiopathogenese des MRS ist bislang ungeklärt. Therapeutisch haben sich Kortikosteroide und Clofazimin bewährt.

Abstract

Melkersson-Rosenthal syndrome is a rare disorder characterized by a triad of recurrent orofacial swelling, recurrent paralysis of the facial nerve and lingua plicata. The complete triad only occurs in 25% of MRS cases. Monosymptomatic or oligosymptomatic variants are more frequent; other findings include granulomas in other facial sites, regional lymphadenopathy, fever, psychotic disorders and hyperplastic gingivitis are associated with MRS. This can be a diagnostic challenge. The etiology and pathogenesis of MRS are unknown. Corticosteroids or clofazimine appear the best therapeutic options.

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Literatur

  1. Apaydin R, Bilen N, Bayramgurler D et al. (2000) Detection of Mycobacterium tuberculosis DNA in a patient with Melkersson-Rosenthal syndrome using polymerase chain reaction. Br J Dermatol 142:1251–1252

    Article  CAS  PubMed  Google Scholar 

  2. Butenschon H (1976) Melkersson-Rosenthal-Syndrom im Kindesalter. Hautarzt 27:544–547

    CAS  PubMed  Google Scholar 

  3. Cockerham KP, Hidayat AA, Cockerham GC et al. (2000) Melkersson-Rosenthal syndrome: new clinicopathologic findings in 4 cases. Arch Opthalmol 118:227–232

    CAS  Google Scholar 

  4. Dummer W, Lurz C, Jeschke R et al. (1999) Granulomatous cheilitis and Crohn’s disease in a 3-year-old boy. Pediatr Dermatol 16:39–42

    Article  CAS  PubMed  Google Scholar 

  5. Dutt SN, Mirza S, Irving RM, Donaldson I (2000) Total decompression of facial nerve for Melkersson-Rosenthal syndrome. J Laryngol Oto 114:870–873

    Article  CAS  Google Scholar 

  6. Gottwald W (1976) Melkersson-Rosenthal-Syndrom (Klinik, Nosologie und Therapie). Dsch Med Wochenschr 101:338–344

    CAS  Google Scholar 

  7. Greene RM, Rogers RS 3rd (1989) Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 21:1263–1270

    CAS  PubMed  Google Scholar 

  8. Hornstein OP (1973) Melkersson-Rosenthal syndrome: a neuro-mucocutaneous disease of complex origin. Curr Probl Dermatol 5:117–156

    CAS  PubMed  Google Scholar 

  9. Iwao F, Sawamura D, Yolota K, Shimizu H (2003) Granulomatous blepharitis successfully treated with tranilast. Br J Dermatol 149:222–224

    Article  CAS  PubMed  Google Scholar 

  10. Kato T, Tagami H (1986) Successful treatment of cheilitis granulomatosa with tranilast. J Dermatol 13:402–403

    CAS  PubMed  Google Scholar 

  11. Liu H, Zheng L, Liu H (2001) Spirochetes-the possible etiological factor of the cheilitis granulomatosa. Chin Med Sci 16:52–55

    CAS  Google Scholar 

  12. Medeiros M, Araujo MI, Guimaraes NS et al. (2002) Therapeutic response to thalodomide in Melkersson-Rosenthal syndrome: a case report. Ann Allergy Immunol 88:421–424

    Google Scholar 

  13. Melkersson E (1928) Case of recurrent facial paralysis with angioneurotic edema. Hygiea 90:737–741

    Google Scholar 

  14. Miescher G (1945) Über essentielle granulomatöse Makrocheilie (Cheilitis granulomatosa). Dermatologica 91:57–85

    Google Scholar 

  15. Neuhofer J, Fritsch P (1984) Cheilitis granulomatosa (Melkersson-Rosenthal-Syndrom): Behandlung mit Clofazimin. Hautarzt 35:459–463

    CAS  PubMed  Google Scholar 

  16. Oliver DW, Scott MJ (2002) Lip reduction cheiloplasty for Miescher’s granulomatous macrocheilitis (Cheilitis granulomatosa) in childhood. Clin Exp Dermatol 27:129–131

    Article  PubMed  Google Scholar 

  17. Olivier V, Lacour JP, Castanet J et al. (2000) Cheilitis granulomatosa in a child. Arch Pediatr 7:274–277

    Article  CAS  PubMed  Google Scholar 

  18. Perez-Calderon R, Gonzalo-Garijo MA, Chaves A, Argila D (2004) Cheilitis granulomatosa of Melkersson-Rosenthal syndrome: treatment with intralesional corticosteroid injections. Allergol Immunopathol 32:36–38

    Article  CAS  Google Scholar 

  19. Rhodes EL, Stirling GA (1965) Granulomatous cheilitis. Arch Dermatol 92:40–44

    Article  Google Scholar 

  20. Ridder GJ, Fradis M, Lohle E (2001) Cheilitis granulomatosa Miescher: treatment with clofazimine and review of the literature. Ann Otol Rhinol Laryngol 110:964–967

    CAS  PubMed  Google Scholar 

  21. Rogers RS (1996) Melkersson-Rosenthal syndrome and orofacial granulomatosis. Dermatol Clin 14:371–379

    PubMed  Google Scholar 

  22. Rogers RS (2000) Granulomatous cheilitis, Melkersson-Rosenthal syndrome, and orofacial granulomatosis. Arch Dermatol 136:1557–1558

    Article  PubMed  Google Scholar 

  23. Rosenthal C (1931) Klinisch-erbbiologischer Beitrag zur Konstitutionspathologie. Gemeinsames Auftreten von (rezidivierender familiärer) Fazialislähmung, angioneurotischem Gesichtsödem und Lingua plicata in Arthritismus-Familien. Z Neurol Psychiatr 131:475–501

    Google Scholar 

  24. Safa G, Joly P, Boullie MC et al. (1995) Melkersson-Rosenthal syndrome treated by thalidomide. 2 cases. Ann Dermatol Venereol 122:609–611

    CAS  PubMed  Google Scholar 

  25. Shapiro M, Peters S, Spinelli HM (2003) Melkersson-Rosenthal syndrome in the periocular area: a review of the literature and a case report. Ann Plast Surg 50:644–648

    Article  PubMed  Google Scholar 

  26. Smeets E, Fryns JP, van den Berghe H (1994) Melkersson-Rosenthal syndrome and de novo autosomal t (9;21) (p11;p11) translocation. Clin Genet 45:323–324

    CAS  PubMed  Google Scholar 

  27. Sussman GI, Yang WH, Steinberg S (1992) Melkersson-Rosenthal syndrome: clinical, pathologic, and therapeutic considerations. Ann Allergy 69:187–194

    CAS  PubMed  Google Scholar 

  28. Sweatman MC, Tasker R, Warner JO et al. (1986) Oro-facial granulomatosis. Response to elemental diet and provocation by food additives. Clin Allergy 16:331–338

    CAS  PubMed  Google Scholar 

  29. Tadokoro T, Ozawa K, Muso Y et al. (2003) Melkersson-Rosenthal syndrome caused by saprodontia: a case report. J Dermatol 30:679–682

    PubMed  Google Scholar 

  30. van de Scheur MR, van der Waal RI, Volker-Dieben HJ et al. (2003) Orofacial granulomatosis in a patient with Crohn’s disease. J Am Acad Dermatol 49:952–954

    Article  PubMed  Google Scholar 

  31. van der Waal RI, Schulten EA, van der Meij EH et al. (2002) Cheilitis granulomatosa: overview of 13 patients with long-term-follow-up-results of management. Int J Dermatol 41:225–229

    Article  PubMed  Google Scholar 

  32. Ziem PE, Pfrommer C, Goerdt S et al. (2000) Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 143:860–863

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Schleswig-Holstein, Kiel

    F. Nagel & R. Foelster-Holst

  2. Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Schleswig-Holstein; Campus Kiel, Brunswiker Straße 10, 24105 , Kiel

    F. Nagel

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Correspondence to F. Nagel.

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Nagel, F., Foelster-Holst, R. Cheilitis granulomatosa. Hautarzt 57, 121–126 (2006). https://doi.org/10.1007/s00105-005-0981-y

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