Orthodontic Treatment in Patients With Epidermolysis Bullosa (EB)-Clinical Practice Guidelines (CPG).

Abstract

<h4>Background</h4>Epidermolysis bullosa (EB) is a rare genetic condition characterized by skin and mucosal fragility. The clinical phenotype is highly variable. Severe types and subtypes, such as junctional EB (JEB), kindler EB (KEB), and recessive dystrophic EB (RDEB), are considered to present a high risk of oral health problems, including malocclusions. Despite this, the literature on orthodontic treatment in patients with EB is scarce and is limited to a few case reports.<h4>Objective</h4>To provide the users with information on the current best practices for orthodontic and dentofacial orthopedic diagnosis and treatment for patients with EB.<h4>Methods</h4>Current information regarding orthodontic treatment in patients with EB was identified based on a systematic literature review. A panel of experts was invited to provide additional information based on their experience through an open-ended form. Later, a Delphi study was performed over two rounds with a consensus threshold at 75%. Members of the medical team and patient representatives revised the final document.<h4>Results</h4>The panel (n = 12) agreed on a total of 15 recommendations, divided into three categories: general information on EB and orthodontics; orthodontic diagnosis and orthodontic treatment. A fourth category on perspectives was developed based on the feedback provided by non-dental members of the medical team (n = 4) and patients (n = 2).<h4>Conclusions</h4>Orthodontic treatment guidelines for patients living with EB are presented, including general aspects of EB, orthodontic diagnosis, and orthodontic treatment.