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Oral Care in Kindler Syndrome: 7-Year Follow-up of 2 Brothers

  • Isabelle Blanchet1,*,
  • Corinne Tardieu1
  • Estelle Casazza1

1UFR Odontologie Aix-Marseille-Université, Service Odontologie, Timone- Assistance Publique Hôpitaux de Marseille, France

DOI: 10.17796/1053-4625-45.1.8 Vol.45,Issue 1,January 2021 pp.41-47

Published: 01 January 2021

*Corresponding Author(s): Isabelle Blanchet E-mail: isabelle.blanchet@univ-amu.fr

Abstract

Background: Kindler poikiloderma is an inherited autosomal genodermatosis characterized by blistering of the epidermis and mucosae. Its prevalence is unknown. Case report: We monitored two brothers suffering from this pathology. Oral manifestations mainly take the form of periodontal lesions. In our patients we noted gingivitis progressing to periodontitis as follow-up care was not effective. We also diagnosed enamel hypoplasia, described more rarely in this pathology. Conclusion: Periodontitis in Kindler Syndrome responds to maintenance therapy, but the absence of surveillance is penalized by a deterioration in periodontal condition and complication of management. All restorative, endodontic, surgical, periodontal and orthodontic treatments should be performed with appropriate precautions.

Keywords

Kindler syndrome; Epidermolysis bullosa; Enamel hypoplasia; Periodontal disease

Cite and Share

Isabelle Blanchet,Corinne Tardieu,Estelle Casazza. Oral Care in Kindler Syndrome: 7-Year Follow-up of 2 Brothers. Journal of Clinical Pediatric Dentistry. 2021. 45(1);41-47.

References

1. Krämer SM, Serrano MC, Zillmann G and al. Oral Health Care for Patients with Epidermolysis Bullosa–Best Clinical Practice Guidelines. Int J Paediatr Dent 22:1-3. 3, Sep 2012.

2. Wiebe CB, Penagos H, Luong N and al. Clinical and microbiologic study of periodontitis associated with Kindler syndrome. J Periodontol 74(1):25-31, Jan 2003.

3. Wright JT, Fine JD, Johnson L. Hereditary epidermolysis bullosa: oral manifestations and dental management. Pediatr Dent 15(4): 242-8, Aug 1993.

4. Budunelli E, Ilgenli T, Buduneli N and al. Acellular dermal matrix allograft used to gain attached gingiva in a case of epidermolysis bullosa. J Clin Periodontol 11(30) :1011 – 1015, 2018.

5. Lindemeyer R, Wadenya R, Maxwell L. Dental, and anaesthetic management of children with dystrophic epidermolysis bullosa. Int J Paediatr Dent 19(2):127-34, Mar 2009.

6. Pacheco W, De Sousa Araugio RM. Orthodontic treatment of a patient with recessive dystrophic epidermolysis bullosa: a case report. Spec Care Dentist 28(4):136-9, Aug 2008.

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