Dental & Oral Health

Introduction: Epidermolysis bullosa is a series of skin disorders characterized by blistering and mechanical fragility of the skin, including the oral mucosa and digestive system lining. Because of its low prevalence, many dentists have limited knowledge of the disease. The scientific literature regarding oral health care of people living with EB is relatively scarce. This makes it difficult for dentists with no experience in treating people with EB to know how to approach them in a safe manner given all the special care these patients might need. DEBRA International entrusted the development of Clinical Guidelines to health care professionals with significant experience in EB around the world. These guidelines contain the appropriate precautions that people with EB might require to receive optimal oral health care. The present guidelines on dental care for people living with EB have been developed by an international team of dentists, using a standard methodology based on a systematic review of the currently available scientific evidence. These guidelines can be applied to all patients diagnosed with epidermolysis bullosa. As such, the guideline considers information for all four major types of EB: EB simplex, junctional EB, dystrophic EB, and Kindler syndrome.

 

Background: A 6-year old girl presented to the Leeds Dental Institute (LDI) for dental treatment.

 

Case Report: Clinical and radiographic examination revealed that she has dental caries affecting both anterior and posterior teeth, early mixed dentition, class 1 malocclusion, and poor oral hygiene. Detailed medical and dental history was obtained from medical consultants and parents. Clinical report of her medical condition was provided by her medical team confirmed she has Dominant Dystrophic EB. Liaison with medical team supervised her case were made to check if there are any special precautions or investigations that need to be undertaken pre, peri and postoperatively in patient’s care. Comprehensive treatment including prevention, restorations of anterior teeth 52, 62, and 73 were carried out using composite resin restorations. Extraction of badly decayed teeth 55, 54, 64, 65, 74 and 84 and all the treatment modifications and precautions were used based on oral health care for patients with epidermolysis bullosa guideline published by DEBRA.

 

Follow-up: She was followed up for 6 months after initial presentation and both parents and patient were satisfied with the outcome.

 

Conclusion: Epidermolysis bullosa is a rare disease with multiple oral manifestations, which requires a special approach from the dental point of view. It is important that dental practitioners who treat patients with EB know how to best manage their care. This research presentation reviews the major causes and manifestations of EB, discusses a unique case study of how dental treatment was rendered to a patient with the condition, and suggests ways to properly manage the oral health needs of patients with EB and similar disorders.