Oral health status in children with inherited dystrophic epidermolysis bullosa

Abstract

Background/Aim. Epidermolysis bullosa is a group of rare, genetic connective tissue diseases that cause blisters in the skin and mucosal membranes. The aim of this study was to evaluate the oral health status of patients with epidermolysis bullosa dystrophica and level of knowledge and opinion of parents about the implementation of preventive measaures and quality of dental care of affected children. Methods. This study included a group of 17 patients from Serbia suffering from dystrophic epidermolysis bullosa and matched control group. Dental caries status was assessed using the Klein-Palmer index. Oral hygiene status was verified with oral hygiene indices, simplified plaque index, and calculus index as described by Green and Vermillion. The gingiva was assessed as healthy or inflamed (gingivitis) on the basis of any changes in color, shape, size and surface texture. The condition of oral mucosa has been registered on the basis of inspection of the lips, tongue, a floor of the mouth, mouth vestibule and palate. The level of knowledge and the impressions of parents about the application of preventive measures were investigated through two questionnaires specifically designed for this study. Results. In both dentitions, there was the highest percentage of caries teeth. In primary dentition average value of the modified plaque index was 1.4 ? 1.14 and modified calculus was 0.7 ? 1. On permanent teeth average plaque index was 2 ? 0.4, and average calculus 1.6 ? 0.6. Statistically, significant higher values were found in permanent dentition in percentage distribution of decayed, missing, filled teeth and also for plaque and calculus indices between affected children and the control group. Most common findings on mucosa were microstomia (76.5%) and ankyloglossia (88.2%). Conclusion. The absence of protocol between the treating physician and the dentist and not sufficiently informed parents are leading to inadequate dental care. The implementation of preventive measures is of most importance to decrease the risk of severe complications that are difficult to be managed.

A systematic literature review of the disease burden in patients with recessive dystrophic epidermolysis bullosa

“…The clinical burden of microstomia (abnormally small oral orifice) was reported in three studies [21,48,50]. [50]; the average maximal mouth opening in this patient cohort was 24.3 mm (SD, 11.6 mm).…”

“…In three studies of neonates with RDEB, the vast majority of infants developed blisters within one week of birth (range of 70% to 94%; Table 1 ) [ 16 , 33 , 35 ]. Five studies reported on the incidence of oral blisters or lesions in patients with RDEB [ 21 , 22 , 33 , 43 , 48 ]. The proportion of patients experiencing these lesions ranged from 79 to 100% [ 33 , 43 , 48 ].…”

“…The clinical burden of microstomia (abnormally small oral orifice) was reported in three studies [ 21 , 48 , 50 ]. A single-center case review of 17 Serbian patients up to age 21 years with DEB (88% RDEB) reported 77% of patients (N = 13) had microstomia, with an average mouth opening capacity of 40.1 mm (SD, 6.6) [ 21 ]. A Spanish multicenter case review found an average oral aperture in RDEB patients (N = 35) of 20.4 mm, compared to an average of 46 mm in healthy controls (N = 45) [ 48 ].…”

“…Other commonly reported symptoms and complications, including nail dystrophy and loss, milia, dental caries, infections, constipation, and musculoskeletal contractures are reported in 12 studies (Table 1 ) [ 16 , 17 , 20 , 21 , 30 , 31 , 33 , 34 , 43 , 52 , 53 , 55 ].…”

“…Additionally, over three-quarters (79%) reported that their private life had suffered and 64% chose not to have more children due to their child's illness. Fifty-nine percent reported that their relationship was negatively affected by their child's illness, and 50% had little 2b) compared to patients with vulvodynia (50 [17]), eczema (48 [23]), dermatomyositis (42 [25]), psoriasis (42 [21]), rosacea (33 [20]), alopecia (31 [24]), and acne vulgaris (30 [19]) as well as people without skin disease (14 [2]); significance not reported Patients with RDEB had the highest emotion scale score (75 [16]; Fig. 2b) compared to patients with vulvodynia (50 [20]), dermatomyositis (45 [27]), eczema (41 [27]), acne vulgaris (41 [25]), psoriasis (39 [27]), rosacea (33 [20]), and alopecia (27 [33]) as well as people without skin disease (9 [13]); significance not reported Patients with RDEB had the highest function scale score (77 [12]; Fig.…”