Ocular Surface Center and Ocular Surface Research & Education Foundation, Miami, FL 33173, USA.
Ocul Surf. 2010 Oct;8(4):193-203. doi: 10.1016/s1542-0124(12)70234-3.
Stevens-Johnson syndrome (SJS) and its more severe variant, toxic epidermal necrolysis (TEN), are relatively rare but have high mortality rates. Survivors are frequently afflicted with severe blinding ocular surface diseases. Recent literature in the areas of ophthalmology and dermatology illustrate that the ophthalmologist's role should no longer be considered secondary in the acute management of SJS/TEN. Accurately differentiating SJS or TEN from erythema multiforme majus (EMM) at the onset of the disease is important, because the management approach to these disease entities differs. Systemic cyclosporine and intravenous immunoglobulin have shown some potential as treatments for SJS/TEN, but their efficacies remain controversial. Amniotic membrane transplantation and intravenous corticosteroid pulse therapy at the acute stage have shown promise for preventing late sight-threatening cicatricial complications. An improved staging system for the ocular surface involvement of acute SJS/TEN, as well as better understanding of the underlying destructive pathogenic mechanism, should further improve our ability to predict ocular involvement and develop new therapeutics to abort destructive processes at the acute stage.
史蒂文斯-约翰逊综合征(SJS)及其更严重的变体中毒性表皮坏死松解症(TEN)较为罕见,但死亡率较高。幸存者常患有严重的致盲性眼表疾病。眼科和皮肤科的近期文献表明,眼科医生在 SJS/TEN 的急性治疗中的作用不应再被视为次要。在疾病发作时准确地区分 SJS 或 TEN 与大疱性多形红斑(EMM)很重要,因为这些疾病实体的治疗方法不同。环孢素和静脉注射免疫球蛋白已显示出对 SJS/TEN 的一定疗效,但疗效仍存在争议。羊膜移植和急性期静脉内皮质类固醇脉冲疗法在预防晚期威胁视力的瘢痕性并发症方面显示出前景。一种用于急性 SJS/TEN 眼表受累的改良分期系统,以及对潜在破坏性发病机制的更好理解,应进一步提高我们预测眼部受累并开发新疗法以在急性期阻止破坏性过程的能力。