Oral Health-related Quality of Life in Patients with Dystrophic Epidermolysis Bullosa

Background: Dystrophic epidermolysis bullosa (DEB) is a devastating mucocutaneous inherited disorder that can have a remarkable impact on the oral cavity. Objective: To understand the relationships between oral health-related quality of life and general quality of life in DEB patients versus a control group. Methods: Twenty-eight DEB patients and 26 healthy individuals completed the following battery of scales: Oral Health Impact Profile (OHIP-49), the RAND Short Form-36 (SF-36), the Hamilton Rating Scale for anxiety (HAM-A) and depression (HAM-D). Results: All 7 dimensions in OHIP-49 demonstrated a statistically significant difference in functional limitations (Median:13.0; IQR:10.3-20.0 vs Median:7.0; IQR:2.0-9.0; p<0.001) and physical disability (Median:11.5; IQR:4.0-15.0 vs Median:0.0; IQR:0.0-2.3; p<0.001) in DEB patients vs controls, respectively. In RAND SF-36, physical functioning and general health were highly significant (p<0.001), whereas limitation due to physical health (p=0.043) and pain (p=0.010) were moderately significant. A positive correlation was found between oral health-related quality of life and anxiety (p=0.005 and p=0.03 in DEB patients and controls, respectively) and depression (p=0.045 and p=0.001 in DEB patients and controls, respectively). In DEB patients, no correlation was found between general quality of life and anxiety and depression, oral health-related quality of life and oral-pharyngeal severity score, number of oral-pharyngeal sites involved and general quality of life, as well as oral health-related quality of life between patients with dominant and recessive DEB forms (p>0.05). Conclusions: The oral health-related quality of life is highly impaired in patients with DEB. General quality of life was affected only for some dimensions and was not significantly correlated with oral health-related quality of life.