Table_1_Anti-p200 Pemphigoid: A Systematic Review.docx

The many clinical aspects of anti-p200 pemphigoid are not well-characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase, and Web of Science databases (1900–2018). Case reports and series of patients were included. A total of 68 eligible studies that comprised 113 anti-p200 pemphigoid patients were included in the qualitative analysis, where there was a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, and 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%), were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than in the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.

抗-p200天疱疮的临床多种表现尚未得到充分描述。本研究旨在分析并关联抗-p200天疱疮的流行病学、临床、组织学及免疫学特征。通过查阅Medline、Embase和Web of Science数据库（1900-2018年）的相关文献，纳入了病例报告及患者系列研究。在定性分析中，共纳入了68项符合条件的研究，涉及113例抗-p200天疱疮患者，平均发病年龄为65.5岁。所有患者均表现为水疱/脓疱，其中54.3%的患者伴有荨麻疹性斑块。66.1%的病例报告了与天疱疮的相似性，但51.4%的患者出现了掌跖部位、40.3%的患者出现了头部和38.5%的患者出现了粘膜部位的受累，此外，频繁出现疤痕/粟丘疹（15.7%）。在73.1%的患者血清中检测到了针对重组层粘连蛋白γ1的自身抗体。28.3%的抗-p200天疱疮患者存在银屑病，尤其是在日本患者中（56.4%），该亚组中脓疱型银屑病的发病率显著高于普通人群。总之，当年轻人群中发生亚表皮自身免疫性大疱病，并伴有明显的指端和头部分布以及粘膜受累时，可怀疑诊断为抗-p200天疱疮。