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Melkersson-Rosenthal Syndrome: Case Reports

Year 2011, Volume: 16 Issue: 4, 215 - 218, 01.08.2011

Hatice Gamze Poyrazoğlu Mehmet Canpolat Hakan Gümüş Hüseyin Per Sefer Kumandaş

Abstract

Melkersson Rosenthal Syndrome is a neuro-mucocutaneous granulomatous disease, characterized by recurrent facial nerve paralysis, orofacial edema and fissured tongue. The cause of Melkersson Rosenthall Syndrome is unknown, but genetic and acquired factors may play a role. This syndrome is very rare in childhood, it is more frequently seen in the second and the third decades of life. Classical triad of this syndrome is very rarely seen. MRS usually occurs as monosymptomatic or oligosymptomatic involment. The presence of two or one of the manifestations with granulomatous cheilitis in the biopsy is sufficient to make the diagnosis of Melkersson-Rosenthal Syndrome. Treatment is symptomatic and may include medical therapies such as nonsteroid anti-inflamatory drugs, steroids and antibiotics. Melkersson-Rosenthal Syndrome should be considered in differential diagnosis of recurrent facial paralysis. Here, we present two girls with classical triad of Melkersson Rosenthall Syndrome.

Keywords

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References

  • 1. Melek H, Köken R, Bükülmez A. ve ark. MelkerssonRosenthal Sendromu: Bir Olgu Sunumu. Güncel Pediatri 2007; 5: 82-84.
  • 2. Markus TE, Gilai A, Mimouni M, Shuper A. Recurrent facial nevre palsy in pediatric patients. Eur J Pediatr 2001; 160: 659- 663.
  • 3. Dodi I, Verri R, Brevi B, et al. Monosymptomatic MelkerssonRosenthal syndrome in an 8-year old boy. Acta Biomed 2006; 77: 20-23.
  • 4. Greco F, Barbagallo M L, Guglielmino R, Sorge G. Recurrent facial nevre palsy associated with anti-GQ1b IgG antibodies. Brain & Development 2008; 30: 606-608
  • 5. Ziem PE, Pfrommer C, Goerdt S, Orfanos CE, Blume-Peytavi U. Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 2000; 143: 860-863.
  • 6. Devriese PP, Schumacher T, Scheide A, de Jongh RH, Houtkooper JM. Incidence, prognosis and recovery of Bell's palsy. A survey of about 1000 patients (1974-1983). Clin Otolaryngol Allied Sci 1990; 15: 15- 27.
  • 7. Van der Waal R, Shulten E, Van de Scheur MR, et al. Cheilitis granülomatoza. J Eur Acad Dermatol Venereol 2001; 15: 519- 523.
  • 8. Perez- Calderon R, Gonzalo- Garijo MA, Chaves A, de Argila D. Cheilitis granulomatosa of Melkersson-Rosenthal syndrome: treatment with intralesional corticosteroid injections. Allergol Immunopathol 2004; 32: 36-38.
  • 9. Ridder GJ, Fradis M, Lohle E. Cheilitis granulomatosa Miescher: treatment with clofazimine and review of the literature. Ann Otol Rhinol Laryngol 2001; 110: 964-967.
  • 10. Kruse-Lösler B, Presser D, Metze D, Joos U. Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Arch Dermatol 2005; 141: 1085-1091.
  • 11. Mahler V, Kiesewetter F. Glossitis granulomatosa symptom of oligosymptomatic Melkersson-Rosenthal syndrome. HNO 1996; 44: 471-475.

Melkersson-Rosenthal Sendromlu: İki Olgu

Year 2011, Volume: 16 Issue: 4, 215 - 218, 01.08.2011

Hatice Gamze Poyrazoğlu Mehmet Canpolat Hakan Gümüş Hüseyin Per Sefer Kumandaş

Abstract

Melkersen Rozenthal sendromu tekrarlayan periferik fasiyal paralizi, orofasiyal ödem ve fissürlü dil triadı ile karakterize nöro-mukokütan granülomatöz bir hastalıktır. Nedeni tam bilinmemekle beraber genetik ve kazanılmış faktörler etiyolojide rol almaktadır. Çocukluk çağında nadir görülen bu sendrom hayatın 2. ve 3. dekadında daha sık görülür. Klasik triadın görülmesi nadirdir ve genellikle monosemptomatik veya oligo semtomatik tutulum izlenir. Bulgulardan bir veya ikisinin varlığında tanı için yüzdeki ödemden cilt biyopsisi yapılarak granülamotöz keilitin varlığının gösterilmesi gerekmektedir. Tedavi semptomatiktir ve medikal tedavi olarak steroid, non steroid anti-inflamatuar ilaçlar ve antibiyotikler kullanılabilir. Melkersson Rosenthal sendromu tekrarlayan fasiyal paralizilerin ayırıcı tanısında düşünülmesi gereken bir hastalıktır. Bu yazıda oldukça nadir görülmesi ve klasik triadın bir arada olması nedeniyle cilt biyopsisine gerek kalmadan Melkersen Rozenthal sendromu tanısı alan iki kız hasta sunulmuştur.

Keywords

Melkersson-Rosenthal sendromu Orofasiyal ödem Tekrarlayan fasiyal paralizi

References

  • 1. Melek H, Köken R, Bükülmez A. ve ark. MelkerssonRosenthal Sendromu: Bir Olgu Sunumu. Güncel Pediatri 2007; 5: 82-84.
  • 2. Markus TE, Gilai A, Mimouni M, Shuper A. Recurrent facial nevre palsy in pediatric patients. Eur J Pediatr 2001; 160: 659- 663.
  • 3. Dodi I, Verri R, Brevi B, et al. Monosymptomatic MelkerssonRosenthal syndrome in an 8-year old boy. Acta Biomed 2006; 77: 20-23.
  • 4. Greco F, Barbagallo M L, Guglielmino R, Sorge G. Recurrent facial nevre palsy associated with anti-GQ1b IgG antibodies. Brain & Development 2008; 30: 606-608
  • 5. Ziem PE, Pfrommer C, Goerdt S, Orfanos CE, Blume-Peytavi U. Melkersson-Rosenthal syndrome in childhood: a challenge in differential diagnosis and treatment. Br J Dermatol 2000; 143: 860-863.
  • 6. Devriese PP, Schumacher T, Scheide A, de Jongh RH, Houtkooper JM. Incidence, prognosis and recovery of Bell's palsy. A survey of about 1000 patients (1974-1983). Clin Otolaryngol Allied Sci 1990; 15: 15- 27.
  • 7. Van der Waal R, Shulten E, Van de Scheur MR, et al. Cheilitis granülomatoza. J Eur Acad Dermatol Venereol 2001; 15: 519- 523.
  • 8. Perez- Calderon R, Gonzalo- Garijo MA, Chaves A, de Argila D. Cheilitis granulomatosa of Melkersson-Rosenthal syndrome: treatment with intralesional corticosteroid injections. Allergol Immunopathol 2004; 32: 36-38.
  • 9. Ridder GJ, Fradis M, Lohle E. Cheilitis granulomatosa Miescher: treatment with clofazimine and review of the literature. Ann Otol Rhinol Laryngol 2001; 110: 964-967.
  • 10. Kruse-Lösler B, Presser D, Metze D, Joos U. Surgical treatment of persistent macrocheilia in patients with Melkersson-Rosenthal syndrome and cheilitis granulomatosa. Arch Dermatol 2005; 141: 1085-1091.
  • 11. Mahler V, Kiesewetter F. Glossitis granulomatosa symptom of oligosymptomatic Melkersson-Rosenthal syndrome. HNO 1996; 44: 471-475.
There are 11 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

Hatice Gamze Poyrazoğlu This is me

Mehmet Canpolat This is me

Hakan Gümüş This is me

Hüseyin Per This is me

Sefer Kumandaş This is me

Publication Date August 1, 2011
Published in Issue Year 2011 Volume: 16 Issue: 4

Cite

APA Poyrazoğlu, H. G., Canpolat, M., Gümüş, H., Per, H., et al. (2011). Melkersson-Rosenthal Sendromlu: İki Olgu. Fırat Tıp Dergisi, 16(4), 215-218.
AMA Poyrazoğlu HG, Canpolat M, Gümüş H, Per H, Kumandaş S. Melkersson-Rosenthal Sendromlu: İki Olgu. Fırat Tıp Dergisi. August 2011;16(4):215-218.
Chicago Poyrazoğlu, Hatice Gamze, Mehmet Canpolat, Hakan Gümüş, Hüseyin Per, and Sefer Kumandaş. “Melkersson-Rosenthal Sendromlu: İki Olgu”. Fırat Tıp Dergisi 16, no. 4 (August 2011): 215-18.
EndNote Poyrazoğlu HG, Canpolat M, Gümüş H, Per H, Kumandaş S (August 1, 2011) Melkersson-Rosenthal Sendromlu: İki Olgu. Fırat Tıp Dergisi 16 4 215–218.
IEEE H. G. Poyrazoğlu, M. Canpolat, H. Gümüş, H. Per, and S. Kumandaş, “Melkersson-Rosenthal Sendromlu: İki Olgu”, Fırat Tıp Dergisi, vol. 16, no. 4, pp. 215–218, 2011.
ISNAD Poyrazoğlu, Hatice Gamze et al. “Melkersson-Rosenthal Sendromlu: İki Olgu”. Fırat Tıp Dergisi 16/4 (August 2011), 215-218.
JAMA Poyrazoğlu HG, Canpolat M, Gümüş H, Per H, Kumandaş S. Melkersson-Rosenthal Sendromlu: İki Olgu. Fırat Tıp Dergisi. 2011;16:215–218.
MLA Poyrazoğlu, Hatice Gamze et al. “Melkersson-Rosenthal Sendromlu: İki Olgu”. Fırat Tıp Dergisi, vol. 16, no. 4, 2011, pp. 215-8.
Vancouver Poyrazoğlu HG, Canpolat M, Gümüş H, Per H, Kumandaş S. Melkersson-Rosenthal Sendromlu: İki Olgu. Fırat Tıp Dergisi. 2011;16(4):215-8.