Oral status and dental treatment needs in patients with Epidermolysis Bullosa - a cross-sectional study.

objectiveTo determine the oral health status and dental treatment needs in a cohort of children and adults with different types of Epidermolysis Bullosa (EB). MATERIALS AND

methodsA cross-sectional study was designed. 101 participants with EB (EB Simplex n = 26, Junctional EB = 6, Dominant Dystrophic EB (DDEB) = 20, Recessive Dystrophic EB (RDEB) = 47, Kindler EB = 2) were assessed including simplified debris index (DI-S), Basic Periodontal Examination (BPE), Decay/Missing/Filled Teeth index (DMFT/deft index) and Orthodontic index of complexity, outcome and need (ICON). Treatment needs were classified into preventive, periodontics, restorative, endodontics, orthodontics, prosthodontics, oral surgery, oral pathology, oral radiology, speech therapy and others. Descriptive and statistical analyses were performed.

resultsThe sample showed a Debris Index of 2.04 ± 0.8, with higher levels in participants with recessive dystrophic EB (2.34 ± 0.7). 15.1% of the total sample (n = 15) showed a BPE value of 3 or 4. DMFT was 11.2 ± 0.1, while 30.1% (n = 28/93) showed an ICON value of 43 or higher. Treatment needs were high, with a median of 3 referrals per patient, with the highest needs in restorative dentistry (n=55, 54.5%), prosthodontics (n=52, 51.5%), and speech/oral function therapy (n=43, 42.6%). By EB type, junctional EB (3.67±0.52) and recessive dystrophic EB (3.34±1.03) showed the highest need for referrals.

conclusionOral health status among people living with EB differed across major types. Patients with recessive dystrophic EB were associated with poorer oral hygiene, a higher caries experience and more missing teeth, whereas those with junctional EB were associated with a higher number of restored teeth. On the opposite, those with simplex EB and dominant dystrophic EB were associated with more favourable oral health indicator. The greatest referral needs were for restorative dentistry, prosthodontics and speech therapy, with higher referral needs observed among EB subtypes with high risk of oral disease (recessive dystrophic EB, junctional EB and Kindler EB). Older age was associated with periodontics and prosthodontics referral needs, while male participants were associated with a higher need for speech therapy. CLINICAL RELEVANCE: This research seems to indicate that patients with recessive dystrophic EB and junctional EB are at high risk of oral disease, requiring referrals to at least 3 different dental specialities. In addition to paediatric dentists, special care dentists and orthodontists, dental teams caring for patients with EB should include specialists in restorative dentistry and prosthodontics, with access to consultants in speech and language therapy, endodontics and periodontics.