Martins Freitas Guilherme, Claure Louise Hernandes, de Andrade Fabiana Antunes, Purim Kátia Sheylla Malta
Department of Health Sciences and Medical School, Positivo University, Curitiba, Paraná, Brazil.
Discov Ment Health. 2025 Jun 3;5(1):83. doi: 10.1007/s44192-025-00209-2.
Epidermolysis bullosa (EB) is a rare, chronic, incurable, multisystemic genetic disorder characterized by skin fragility, resulting in blistering from minimal trauma or spontaneously. Despite its physical impact, little is known about the mental health and quality of life (QoL) of those affected. This study aims to investigate these aspects in individuals with EB.
A cross-sectional study was conducted in Brazil using a self-administered questionnaire and a convenience sample. Data on clinical-epidemiological characteristics, QoL (DLQI-BRA and QoLEB), and depression indicators (PHQ-9) were collected. Descriptive statistics and the Chi-square test were used, with a significance level of p < 0.05.
The sample consisted of 31 patients, predominantly women (89.3%), with a mean age of 33.32 ± 11.3 years. The recessive dystrophic subtype was most common (54.8%), and depressive symptoms were present in 71%. PHQ-9 results revealed 29% had "mild depression," 16.1% "moderate depression," 22.6% "moderately severe depression," and 3.2% "severe depression." QoLEB indicated significant impairment in functional domains, while DLQI showed that 45.2% of patients experienced severe QoL impact. The most affected domains were leisure, symptoms and feelings, and daily activities.
The findings highlight the substantial impact of EB on mental health, QoL, and personal relationships. These results reinforce the need for early mental health screening and the integration of multidisciplinary care to optimize patient outcomes. Implementing structured psychological support, along with comprehensive dermatological and medical management, could mitigate the burden of the disease and enhance the overall well-being of affected individuals.
大疱性表皮松解症(EB)是一种罕见的、慢性的、无法治愈的多系统遗传性疾病,其特征为皮肤脆弱,即使受到轻微创伤或自发地也会出现水疱。尽管它对身体有影响,但对于受影响者的心理健康和生活质量(QoL)却知之甚少。本研究旨在调查EB患者的这些方面。
在巴西进行了一项横断面研究,使用自填式问卷和便利样本。收集了临床流行病学特征、生活质量(DLQI-BRA和QoLEB)以及抑郁指标(PHQ-9)的数据。使用描述性统计和卡方检验,显著性水平为p < 0.05。
样本包括31名患者,主要为女性(89.3%),平均年龄为33.32 ± 11.3岁。隐性营养不良亚型最为常见(54.8%),71%的患者存在抑郁症状。PHQ-9结果显示,29%有“轻度抑郁”,16.1%有“中度抑郁”,22.6%有“中度严重抑郁”,3.2%有“重度抑郁”。QoLEB表明功能领域有显著损害,而DLQI显示45.2%的患者生活质量受到严重影响。受影响最大的领域是休闲、症状和感受以及日常活动。
研究结果突出了EB对心理健康、生活质量和人际关系的重大影响。这些结果强化了早期心理健康筛查以及整合多学科护理以优化患者结局的必要性。实施结构化心理支持,以及全面的皮肤病学和医疗管理,可以减轻疾病负担,提高受影响个体的整体幸福感。