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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.

The psychological aspect in patients with dystrophic epidermolysis bullosa (DEB) is poorly documented. We sought to determine the role of DEB in anxiety, depression and self-esteem. We conducted a cross-sectional study, collecting data from 27 DEB patients and 26 healthy individuals. DEB patients and healthy controls completed three different psychometric scales for anxiety and depression and one scale for self-esteem. DEB patients and healthy controls were homogeneous for age and sex (P > 0.05), but not for employment, marital status and economic level (P < 0.05). Median values of all psychometric battery scales were not statistically significant between DEB patients and healthy controls, except for Goldberg scale for anxiety (P = 0.003) and depression (P = 0.037) and slightly significant for Zung Scale for anxiety (P = 0.048) with no difference between DEB patients with dominant versus recessive form in all scales (P > 0.05). Among DEB patients, only employment showed a significant difference in all scales (P < 0.05) but Hamilton for depression, whereas self-esteem seemed to be affected by marriage (P = 0.04) and education (P = 0.016). DEB patients apparently are not more anxious and/or depressed and do not have less self-esteem than healthy individuals.

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.