大疱性表皮松解症(EB)和自身免疫性起泡疾病(AIBD)描述了一组罕见的慢性皮肤病,其特征是皮肤脆性和起泡。虽然不常见,两者均可能发生严重的眼表疾病(OSD),需要进行眼科评估。疾病评分系统在提供疾病严重程度的客观和准确评估方面具有关键作用。这份报告的目的是,首先,记录EB/AIBD眼部受累的患病率和严重程度。其次,回顾和评估现有的EB/AIBD眼部和全身评分系统。最后,确定可在EB/AIBD中进一步开发眼部特异性工具的领域。
2017年10月,利用Medline进行了文献检索,Embase,和Scopus数据库。结果受到公布日期的限制,在01.01.1950和31.10.2017之间。然后审查了这些文章的参考列表,以获得其他相关出版物。如果有英文翻译,则包括所有语言的文章。如果文章是重复的,则被排除在外,没有提到EB/AIBD的眼部受累或描述的其他疾病的眼部受累。
在88篇同行评审的期刊文章中确定了EB/AIBD眼部受累的描述。报告的发现包括但不限于:结膜炎,睑板腺功能障碍,干眼症,倒车灯,symblepharon,穹窿纤维化,角膜病变,外翻/内翻,脚踝,角膜溃疡,视力障碍和失明。尽管存在用于评估粘膜类天疱疮中OSD的评分系统,对于其他AIBD亚型或EB,不存在此类工具。皮肤病学文献中存在几种系统评分系统,可有效地对总体EB/AIBD严重程度进行分级,但有限地包含了眼部特征。据我们所知,没有公认或验证的评分系统可以对EB/AIBD中的眼部表现进行全面的分级或分级.
在EB和AIBD中记录了一系列眼部并发症。为EB/AIBD开发一个综合的眼部评分系统,该系统结合了“活动”和“损害”之间的划分,将有助于更客观的患者评估,改进纵向监测,干预结果的比较,并为这些患者的讨论提供共性,因为他们的护理具有多学科性质。
Epidermolysis bullosa (EB) and autoimmune blistering diseases (AIBD) describe a group of rare chronic dermatoses characterized by cutaneous fragility and blistering. Although uncommon, significant ocular surface disease (OSD) may occur in both and require ophthalmological assessment. Disease scoring systems have a critical role in providing objective and accurate assessment of disease severity. The objectives of this report were, firstly, to document the prevalence and severity of ocular involvement in EB/AIBD. Secondly, to review and evaluate existing ocular and systemic scoring systems for EB/AIBD. Finally, to identify areas where further development of ocular specific tools in EB/AIBD could be pursued.
A literature search was performed in October 2017 utilising Medline, Embase, and Scopus databases. The results were restricted by date of publication, between 01.01.1950 and 31.10.2017. The reference lists of these articles were then reviewed for additional relevant publications. Articles of all languages were included if an English translation was available. Articles were excluded if they were duplicates, had no reference to ocular involvement in EB/AIBD or described ocular involvement in other diseases.
Descriptions of ocular involvement in EB/AIBD were identified in 88 peer-reviewed journal articles. Findings reported include but are not limited to: cicatrising conjunctivitis, meibomian gland dysfunction, dry eye disease, trichiasis, symblepharon, fornix fibrosis, keratopathy, ectropion/entropion, ankyloblepharon, corneal ulceration, visual impairment and blindness. Although scoring systems exist for assessment of OSD in mucous membrane pemphigoid, no such tools exist for the other AIBD subtypes or for EB. Several systemic scoring systems exist in the dermatological literature that are efficacious in grading overall EB/AIBD severity, but have limited inclusion of ocular features. To the best of our knowledge, there is no recognised or validated scoring systems which comprehensively stages or grades the spectrum of ocular manifestations in EB/AIBD.
There are a range of ocular complications documented in EB and AIBD. Development of a comprehensive ocular scoring system for EB/AIBD which incorporates the delineation between \'activity\' and \'damage\' would facilitate more objective patient assessment, improved longitudinal monitoring, comparison of intervention outcomes, and provide commonality for discussion of these patients due to the multidisciplinary nature of their care.