Salamon Gudrun, Strobl Sophie, Matschnig Marie-Stephanie, Diem Anja
HEALTH Lab, Faculty of Psychology, Sigmund Freud University, Vienna, Austria.
EB House Austria, Department of Dermatology and Allergology, University Hospital of the Paracelsus Medical University, Salzburg, Austria.
Orphanet J Rare Dis. 2025 Jan 6;20(1):3. doi: 10.1186/s13023-024-03475-5.
Epidermolysis bullosa (EB) is a serious, painful, hereditary and still incurable genetic condition. Due to blistering or wounds on the skin caused by the slightest touch, a person suffering from epidermolysis bullosa is prevented from achieving the same quality of life as a healthy person. Until now, psychosocial research has focused on the description of the problems of people living with the disease.
The aim of this paper is to provide a structured overview of potential psychosocial effects of epidermolysis bullosa on the everyday lives of people with the condition and to explore helpful aspects for coping with EB.
Semi-structured interviews with persons living with EB were conducted. Analyses were based on a combination of a reflexive grounded theory approach and a structured coding guide. By means of purposive sampling across three countries, a high diversity within the sample was achieved in order to obtain a wide range of possible effects.
A total of 17 individuals living with EB across all EB types were interviewed, resulting in 36,315 words being analysed. Psychosocial aspects of EB comprise physical, emotional, social, and functional dimensions. Identified burdens and helpful aspects in dealing with EB are described along this structure.
Our results highlight the broad range of possible psychosocial effects caused by epidermolysis bullosa. It is particularly important to recognise those affected as individuals with their personal needs and to avoid unnecessary strains. Furthermore, emotional support is crucial in every respect.
大疱性表皮松解症（EB）是一种严重、痛苦、遗传性且仍无法治愈的基因疾病。由于哪怕最轻微的触碰都会导致皮肤起泡或受伤，患有大疱性表皮松解症的人无法拥有与健康人相同的生活质量。到目前为止，社会心理研究主要集中在描述该疾病患者所面临的问题。
本文旨在系统概述大疱性表皮松解症对患者日常生活可能产生的社会心理影响，并探索应对EB的有益方面。
对EB患者进行了半结构化访谈。分析基于反思性扎根理论方法和结构化编码指南的结合。通过在三个国家进行目的抽样，样本具有高度多样性，以获得广泛的可能影响。
共访谈了所有EB类型的17名患者，分析了36315个单词。EB的社会心理方面包括身体、情感、社会和功能维度。沿着这一结构描述了在应对EB时所确定的负担和有益方面。
我们的结果凸显了大疱性表皮松解症可能造成的广泛社会心理影响。特别重要的是，要将受影响者视为有个人需求的个体，并避免不必要的压力。此外，情感支持在各个方面都至关重要。
