Search

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), RAND Short Form-36 (SF-36), Hamilton Rating Scale for anxiety (HAM-A) and depression (HAM-D). Results: All 7 dimensions in OHIP-49 demonstrated significant difference in functional limitations (Median:13.0; IQR:10.3-20.0 vs Median:7.0; IQR:2.0-9.0; p0.05). 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 correlated with oral health-related quality of life.

BACKGROUND: Gingival lesions in patients with dystrophic epidermolysis bullosa (DEB) are a common manifestation. However, their clinical features, frequency and severity are currently unknown. METHODS: Forty-five DEB patients were assessed by an oral medicine specialist, who analysed the presence/absence of four clinical signs (erythema, erosion/ulcer, atrophy, blister) on free and attached gingiva, using the Epidermolysis Bullosa Oropharyngeal Severity score. RESULTS: Twenty-eight (62.2%) out of 45 DEB patients showed different types of gingival lesions, whose presence/absence and total frequency/distribution were not significantly different between males and females (p = 0.087 and p = 0.091, respectively). Erythema was the most prevalent lesion (66.2%) and the recessive DEB severe generalized (RDEB-sev gen) reached the highest median disease activity score. A significant correlation was observed between the DEB subtypes and the disease activity median score (p < 0.001), but not between age and total disease activity score in each group of DEB (p > 0.05). Lastly, logistic regression showed that only gender (p = 0.031) and RDEB-sev gen (p = 0.001) were risks factors for the presence of gingival lesions. CONCLUSIONS: Gingival lesions in DEB patients are a relatively common entity and may have multiple clinical aspects, emphasizing the need for thorough attention and awareness among dentists. 2015 Australian Dental Association.