The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB
The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB
复制标题
DOI：
10.1016/j.jaad.2008.02.004
复制
发表时间：
2008-06-01
影响因子：
13.8
通讯作者：
Zambruno, Giovanna
中科院分区：
文献类型：
--
作者：
Fine, Jo-David;Eady, Robin A. J.;Zambruno, Giovanna
背景：自2000年《大疱性表皮松解症的诊断和分类第二份国际共识报告》发表以来，我们对这类疾病的认识在临床和分子上都取得了许多进展。与此同时，新的大疱性表皮松解症（EB）亚型已被描述，并与其他一些疾病有相似之处。目的：我们寻求对EB亚型的分类达成新的共识。结果：我们现在提出了一个修订的分类系统，考虑到新的进展，以及包括其他遗传性疾病，也应该包括在EB谱中，基于起泡和机械脆弱性的存在。目前的建议是使用特定的诊断测试，并提供了在每个主要EB亚型中已知发现的最新情况。还提供了对临床医生及其患者特别有益的信息和实验室资源的电子链接。局限性：随着对这种疾病的了解越来越多，未来可能需要修改。分级系统的设计对这些修改具有足够的灵活性。结论：修订后的分类系统有助于临床医生准确诊断和分型EB患者。
Background: Since publication in 2000 of the Second International Consensus Report on Diagnosis and Classification of Epidermolysis Bullosa, many advances have been made to our understanding of this group of diseases, both clinically and molecularly. At the same time, new epidermolysis bullosa (EB) subtypes have been described and similarities with some other diseases have been identified.Objective: We sought to arrive a, a new consensus of he classification of EB subtypes.Results: We now present a revised classification system that takes into account the new advances, as well as encompassing other inherited diseases that should also be included within the EB spectrum, based on the presence of blistering and mechanical fragility. Current recommendations are made on the use of specific diagnostic tests, with updates on the findings known to occur within each of the major EB subtypes. Electronic links are also provided to informational and laboratory resources of particular benefit to clinicians and their patients.Limitations: As more becomes known about this disease, future modifications may be needed. The classification system has been designed with Sufficient flexibility for these modifications.Conclusion: This revised classification system should assist clinicians in accurately diagnosing and Subclassifying patients with EB.