Vol. 5 (03) pp. 861-867 DOI: 10.21474/IJAR01/3582

Oral findings in Zinsser-Engman-Cole syndrome: a case report.

, , and
  • Department of Paediatric Dentistry, dental college, University Hassan 2 Casablanca , Morocco.
  • Department of Oral Surgery, dental college, University Hassan 2 Casablanca , Morocco.
  • Department of Paediatrics, Faculty of Medicine, University Hassan 2 Casablanca , Morocco.
13 Downloads 153 Views
Crossref

Download Full Paper (PDF)

Abstract

Zinsser Cole Engmansyndrome also known as dyskératosiscongenita (DC) is a very rare genetic affection characterized by the association ofreticular hyper pigmentation, nail dystrophy and leucoplakia of the mucous membranes. We report the case of a 14 years old child already diagnosed with DC and which was complaining about extensive caries, and teeth loss. Clinical examination revealed a poor oral hygiene, extensive caries and periodontal disease. DC is a inherited bone marrow failure characterized by excessively short telomeres in highly proliferative tissues. Its prevalence is estimated at one in a million.DC has many clinical features and oral manifastations. It have a poor prognosis and its current treatment remain non satisfactory. DC needs a multidisciplinary approach and a regular follow-up.

How to Cite This Article

BADRE. Bouchra, NAJI. Youssef, BOUSFIHA. Aziz and EL ARABI. Samira. (2017); Oral findings in Zinsser-Engman-Cole syndrome: a case report., International Journal of Advanced Research (IJAR), 5 (03), 861-867, ISSN 2320-5407. DOI: https://doi.org/10.21474/IJAR01/3582

Corresponding Author

Naji Youssef
Department of Oral Surgery, dental college, University Hassan 2 Casablanca , Morocco

Article Analytics

References

  1. Brown C. J. Dyskeratosiscongenita: report of a case.International Journal of Paediatric Dentistry.2000;10(4):328–334.
  2. Atkinson J. C., Harvey K. E., Domingo D. L., Trujillo M. I., Guadagnini J.-P., Gollins S., Giri N., Hart T. C., Alter B. P. Oral and dental phenotype of dyskeratosiscongenita.Oral Diseases. 2008;14(5):419–427
  3. Mine Koruyucu,* PelinBarlak, and FigenSeymen. Oral and Dental Findings of DyskeratosisCongenita.  Case Rep Dent. 2014; 2014: 454128.
  4. Monica Bessler,David B. Wilson, and Philip J. MasonDyskeratosiscongenitaFEBS Lett. 2010 Sep 10; 584(17): 3831–8.
  5. Dokal I.DyskeratosisCongenita. American Society of Hematology; 2011.
  6. Davidovich E., Eimerl D., Aker M., Shapira J., Peretz B. Dyskeratosiscongenita: dental management of a medically complex child.Pediatric Dentistry. 2005;27(3):244–248
  7. Zinsser F. Atrophia cutis reticularis cum pigmentatione, dystrophia et leukoplakiaoris.Ikonogr Dermatol.1910;5:219–223.
 
Creative Commons Attribution 4.0 This work is licensed under a Creative Commons Attribution 4.0 International License.