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Dental Approach to Incortinentia Pigmenti- “Bloch-Sulzberger” Syndrome A Case Report

Year 2009, Volume: 36 Issue: 1 - Volume: 36 Issue: 1, 47 - 50, 01.02.2009

Abstract

Incontinentia pigmenti IP is an X-linkeddominant genetic syndrome. It is predominantlyobserved in female 95 % and the few males generally die soon after birth. This syndrome is observedalong with ectodermal disorders. Skin pigmentations are the most common characteristics of IP. IPcauses ectodermal, mesodermal, neurological, ocular and dental abnormalities. The aim of this casepresentation is to document and assess the oral anddental manifestations of a 7 year old female IPpatient. Clinical and radiological dental examination of the patient showed absence of primary andpermanent dental follicles, delayed eruption andsome teeth abnormalities

References

  • Bentolila R., Rivera H., Sanchez-Quevedo M.C. Incontinentia Pigmenti: A case report. Pediatr Dent. 2006; 28: 54-7.
  • Dominguez A.General and dental characte- ristics of Bloch-Sulzberger syndrome. Review of li- terature and presentation of a case report. Medicina Oral 2002; 7: 293-7.
  • Tracey A., Richard R. Incontinentia Pigmenti (Bloch-Sulzberger syndrome): Report of case. J Dent Child. 1999; 36;860 - 2 .
  • Joseph V., Matheson J. Incontinentia Pigmenti (Bloch-Sulzberger syndrome): A case re- port. Oral Surg Oral Med Oral Pathol. 2007; 71: 454- 6.
  • Philip E., Thomas J., Robert D., Harrison. A dentofacial deformity associated with İncontinentia Pigmenti: Report of a case. The University of Chicago Medical Center.
  • Russel DL., Finn SB. İncontinentia Pigmenti (Bloch-Sulzberger syndrome): A case report with emphasis on dental manifestations. J Dent Child. 1967; 34: 494-500.
  • Anil Govindrao Ghom.Textbook of oral medicine.Jaypee brothers medical publishers Ltd 717, 2005.
  • Cho SY, Lee CK, Drummond BK. Surviving male with incontinentia pigmenti:A case report.Int J Pediat Dentist 2004; 14: 69-72.
  • Minic S, Novotny G, Trpinac D, Obradovic M. Clinical features of incontinentia pigmenti with emphasis on oral and dental abnormalities. Clin Oral Invest 2006;10: 343-7.
  • Smahi A, Courtois G, Rabia SH.The NF- kappaB signaling pathway in human diseases from incontinentia pigmenti to ectodermal dysplasis and immune-deficiency syndroms. Human Molecular Genetics 2002;11:2371-5.

İnkontinenti pigmenti- “Bloch-Sulberger Sendromu” vakasına dental yaklaşım olgu sunumu

Year 2009, Volume: 36 Issue: 1 - Volume: 36 Issue: 1, 47 - 50, 01.02.2009

Abstract

İnkontinenti pigmenti İP X genine bağlıdominant geçişli genetik bir sendromdur. Yaygınolarak kızlarda gözlenen % 95 bu hastalık erkeklerde genellikle ölümcüldür. Bu sendrom ektodermal bozukluklarla beraber görülür. Deri pigmentasyonları İP’ nin ilk görülen ve en karakteristik özelliğidir. Bunun dışında nörolojik, oküler, dentalbozukluklara sebep olabilir. Bu vaka sunumununamacı Ankara Üniversitesi Diş Hekimliği Fakültesi’ne ebeveyni tarafından getirilen 7 yaşındaki kızİP hastasında gözlenen oral ve dental bozuklukların belirlenerek değerlendirilmesidir. Hastanın dental klinik ve radyografik muayenesi sonucunda baz› süt ve daimi diş germlerinin gelişmediği, sürme gecikmelerinin bulunduğu, ağızdaki mevcut dişlerde ise şekil anomalileri olduğu saptanmıştır.

References

  • Bentolila R., Rivera H., Sanchez-Quevedo M.C. Incontinentia Pigmenti: A case report. Pediatr Dent. 2006; 28: 54-7.
  • Dominguez A.General and dental characte- ristics of Bloch-Sulzberger syndrome. Review of li- terature and presentation of a case report. Medicina Oral 2002; 7: 293-7.
  • Tracey A., Richard R. Incontinentia Pigmenti (Bloch-Sulzberger syndrome): Report of case. J Dent Child. 1999; 36;860 - 2 .
  • Joseph V., Matheson J. Incontinentia Pigmenti (Bloch-Sulzberger syndrome): A case re- port. Oral Surg Oral Med Oral Pathol. 2007; 71: 454- 6.
  • Philip E., Thomas J., Robert D., Harrison. A dentofacial deformity associated with İncontinentia Pigmenti: Report of a case. The University of Chicago Medical Center.
  • Russel DL., Finn SB. İncontinentia Pigmenti (Bloch-Sulzberger syndrome): A case report with emphasis on dental manifestations. J Dent Child. 1967; 34: 494-500.
  • Anil Govindrao Ghom.Textbook of oral medicine.Jaypee brothers medical publishers Ltd 717, 2005.
  • Cho SY, Lee CK, Drummond BK. Surviving male with incontinentia pigmenti:A case report.Int J Pediat Dentist 2004; 14: 69-72.
  • Minic S, Novotny G, Trpinac D, Obradovic M. Clinical features of incontinentia pigmenti with emphasis on oral and dental abnormalities. Clin Oral Invest 2006;10: 343-7.
  • Smahi A, Courtois G, Rabia SH.The NF- kappaB signaling pathway in human diseases from incontinentia pigmenti to ectodermal dysplasis and immune-deficiency syndroms. Human Molecular Genetics 2002;11:2371-5.
There are 10 citations in total.

Details

Primary Language Turkish
Journal Section Case Report
Authors

Bengi Öztaş This is me

Şebnem Kurşun This is me

Kıvanç Kamburoğlu This is me

Semiha Esirgen This is me

Publication Date February 1, 2009
Published in Issue Year 2009 Volume: 36 Issue: 1 - Volume: 36 Issue: 1

Cite

Vancouver Öztaş B, Kurşun Ş, Kamburoğlu K, Esirgen S. İnkontinenti pigmenti- “Bloch-Sulberger Sendromu” vakasına dental yaklaşım olgu sunumu. EADS. 2009;36(1):47-50.