Shah Amit Aakash, Seiffert-Sinha Kristina, Sirois David, Werth Victoria P, Rengarajan Badri, Zrnchik William, Attwood Kristopher, Sinha Animesh A
Department of Dermatology, University at Buffalo and Roswell Park Cancer Institute, Buffalo, USA.
Acta Derm Venereol. 2015 Jan;95(1):86-90. doi: 10.2340/00015555-1854.
Pemphigus vulgaris (PV) is a rare, potentially life threatening, autoimmune blistering skin disease. The International Pemphigus and Pemphigoid Foundation (IPPF) has recently developed a disease registry with the aim to enhance our understanding of autoimmune bullous diseases with the long-term goal of acquiring information to improve patient care. Patients were recruited to the IPPF disease registry through direct mail, e-mail, advertisements, and articles in the IPPF-quarterly, -website, -Facebook webpage, and IPPF Peer Health Coaches to complete a 38-question survey. We present here the initial analysis of detailed clinical information collected on 393 PV patients. We report previously unrecognized gender differences in terms of lesion location, autoimmune comorbidity, and delay in diagnosis. The IPPF disease registry serves as a useful resource and guide for future clinical investigation.
寻常型天疱疮（PV）是一种罕见的、可能危及生命的自身免疫性水疱性皮肤病。国际天疱疮和类天疱疮基金会（IPPF）最近建立了一个疾病登记处，旨在增进我们对自身免疫性大疱性疾病的了解，其长期目标是获取信息以改善患者护理。通过直邮、电子邮件、广告以及IPPF季刊、网站、脸书网页上的文章和IPPF同伴健康教练招募患者，让他们完成一份包含38个问题的调查问卷。我们在此展示对393例PV患者收集的详细临床信息的初步分析。我们报告了在皮损部位、自身免疫性合并症和诊断延迟方面此前未被认识到的性别差异。IPPF疾病登记处可作为未来临床研究的有用资源和指南。
