Purpose

A review of the published literature on the history, pathogenesis, and treatment of pemphigus vulgaris (PV) and its ocular involvement.

Methods

Literature searches were conducted in MEDLINE (Ovid), and google scholar for pemphigus vulgaris and ocular PV. Inclusion criteria were given to meta-analysis, case-controlled studies, and documented case reports. The data were examined and independently analyzed by more than two of the authors.

Results

PV is a humoral autoimmune disease with a preponderance of IgG4 anti-desmoglein 3 antibodies. Upon antibody binding, there is an intracellular signaling mechanism that leads to blister formation. Ocular findings are seen in up to 16% of PV patients with conjunctivitis being the most common clinical presentation. New steroid-sparing agents have helped with the control of this deadly disease, and with better understanding of the pathogenesis of PV, other cytokine blockers currently available are promising steroid-sparing agents.

Conclusions

Ocular pemphigus can occasionally present prior to mucocutaneous findings. Recalcitrant conjunctivitis with conjunctival blisters should warrant a workup for systemic PV.

中文翻译：

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寻常性天疱疮的眼表受累：一项跨学科综述。

目的

有关寻常型天疱疮（PV）及其眼受累的历史，发病机制和治疗的已发表文献的综述。

方法

文献搜索是在MEDLINE（Ovid）中进行的，而Google学者针对寻常性天疱疮和眼PV进行了搜索。纳入标准用于荟萃分析，病例对照研究和成文的病例报告。两名以上的作者对数据进行了检查和独立分析。

结果

PV是一种体液性自身免疫疾病，主要存在IgG4抗桥粒芯蛋白3抗体。抗体结合后，存在导致泡罩形成的细胞内信号传导机制。在多达16％的PV结膜炎患者中可以看到眼部检查结果，这是最常见的临床表现。新的类固醇保护剂有助于控制这种致命疾病，并且通过更好地了解PV的发病机理，目前可用的其他细胞因子阻滞剂是有希望的类固醇保护剂。

结论

眼天疱疮有时可在皮肤粘膜发现之前出现。顽固性结膜炎伴结膜水泡应检查全身性PV。