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

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.

Kindler syndrome, epidermolysis bullosa,, enamel hypoplasia, periodontal disease

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.