Khan M T, O'Sullivan M, Faitli B, Mellerio J E, Fawkes R, Wood M, Hubbard L D, Harris A G, Iacobaccio L, Vlahovic T, James L, Brains L, Fitzpatrick M, Mayre-Chilton K
Br J Dermatol. 2020 Mar;182(3):e89-e114. doi: 10.1111/bjd.18820.
Epidermolysis bullosa (EB) is a complex rare condition that affects the skin and many parts of the body. Those born with EB have skin so fragile they are called 'butterfly children', their skin is quite simply as fragile as the wing of a butterfly. In the UK it is estimated that there are more than 5,000 people living with EB and 500,000 worldwide. Little clinical guidance for care existed until DEBRA International started a programme to develop clinical practice guidelines (CPGs). There were no previous guidelines and few published studies on foot care in EB so treatment decisions were largely based on individual opinion and experience. The panel - made up of clinical experts and people living with EB representing Australia, the UK, and the USA - aimed to describe foot problems in people of all ages with EB, and summarise current evidence and management. The authors used a logical podiatric (foot) care literature review focussed on patients with EB. The authors found that the evidence in this area was limited but several interventions (treatments) currently practised by podiatrists show positive outcomes. The study allowed the group to make recommendations on how to treat foot and nail disorders in patients with EB. Furthermore, the authors concluded that further research is needed. This is a summary of the study: Foot care in epidermolysis bullosa: evidence-based guideline.
大疱性表皮松解症（EB）是一种复杂的罕见病症，会影响皮肤及身体的许多部位。患有EB的人皮肤极为脆弱，被称为“蝴蝶宝贝”，他们的皮肤就像蝴蝶翅膀一样脆弱不堪。据估计，英国有超过5000人患有EB，全球则有50万人。在国际大疱性表皮松解症患者协会（DEBRA International）启动制定临床实践指南（CPG）项目之前，几乎没有关于护理的临床指导。此前没有针对EB足部护理的指南，也鲜有相关发表研究，因此治疗决策很大程度上基于个人观点和经验。该专家小组由来自澳大利亚、英国和美国的临床专家以及EB患者组成，旨在描述各年龄段EB患者的足部问题，并总结当前的证据及管理方法。作者采用了针对EB患者的逻辑性足病护理文献综述。作者发现该领域的证据有限，但足病医生目前实施的几种干预措施（治疗方法）显示出了积极效果。这项研究使该小组能够就如何治疗EB患者的足部和指甲疾病提出建议。此外，作者得出结论，还需要进一步研究。这是该研究的总结：大疱性表皮松解症的足部护理：循证指南。
