Oral health care pathways for patients with epidermolysis bullosa: A position statement from the European reference network for rare skin diseases.

BACKGROUND

Inherited epidermolysis bullosa (EB) comprises a group of genetic disorders characterized by skin fragility and unique oral features. It requires interdisciplinary care from several health professionals, including oral health teams. Modern dentistry encompasses a wide range of therapeutic options performed by specialists from different fields.

OBJECTIVE

To guide clinicians caring for patients with different types of EB to seek care from different dental services.

METHODS

Dental treatment needs for patients with EB were identified based on a systematic literature review. A panel of experts was consulted and invited to provide additional information through an open-ended question over 7 months. A Delphi study was applied over two rounds to the resulting pathways design. The threshold of consensus was set a priori at 75%. Patients' representatives revised the final document.

RESULTS

The panel (n = 17) agreed on a total of 55 recommendations divided into six groups according to the severity of oral compromise in EB (52 recommendations were agreed on in round 1, and three were agreed on in round 2).

CONCLUSIONS

Dental care pathways are presented for each type of EB. Specific considerations are discussed according to clinical features, including age of first referral, frequency of follow-up appointments, and list of dental specialties involved in the care of patients with EB.