Araki Yayoi, Sotozono Chie, Inatomi Tsutomu, Ueta Mayumi, Yokoi Norihiko, Ueda Eiichiro, Kishimoto Saburo, Kinoshita Shigeru
Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Am J Ophthalmol. 2009 Jun;147(6):1004-11, 1011.e1. doi: 10.1016/j.ajo.2008.12.040. Epub 2009 Mar 14.
To evaluate the visual prognosis of patients with Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), followed by general and topical high-dose corticosteroids administration from disease onset.
Prospective, observational case series.
Between May 1, 2003 and June 30, 2005, we enrolled 5 patients with SJS or TEN with ocular complications at the acute stage. Intravenous pulse therapy with methylprednisolone (steroid pulse therapy; 500 or 1000 mg/day for 3 to 4 days) was initiated within 4 days from disease onset. Topically, 0.1% betamethasone was applied over 5 times daily for at least 2 weeks. Visual acuity (VA) and slit-lamp microscopic appearance 1 year from disease onset were evaluated.
At the first examination, corneal or conjunctival epithelial defects and pseudomembranous conjunctivitis were present in all cases. Skin eruptions dramatically improved after steroid pulse therapy. Although ocular inflammation increased for several days, pseudomembranes disappeared and corneal and conjunctival epithelium regenerated within 6 weeks. At the chronic stage, all eyes had clear corneas with the palisades of Vogt (POV), implying the presence of corneal epithelial stem cells. Best-corrected VA was 20/20 or better in all eyes. Five eyes showed superficial punctate keratopathy. No eye had cicatricial changes except for 1 with slight fornix shortening. No significant adverse effects of steroid occurred during all clinical courses.
Steroid pulse therapy at disease onset is of great therapeutic importance in preventing ocular complications. Topical betamethasone also shows great promise for preventing corneal epithelial stem cell loss in the limbal region and cicatricial changes.
评估从疾病发作起接受全身及局部大剂量皮质类固醇治疗的史蒂文斯-约翰逊综合征（SJS）及其严重变型中毒性表皮坏死松解症（TEN）患者的视力预后。
前瞻性观察病例系列。
在2003年5月1日至2005年6月30日期间，我们纳入了5例急性期伴有眼部并发症的SJS或TEN患者。在疾病发作后4天内开始静脉注射甲泼尼龙进行脉冲治疗（类固醇脉冲疗法；500或1000毫克/天，持续3至4天）。局部应用0.1%倍他米松，每日5次以上，至少持续2周。评估疾病发作1年后的视力（VA）和裂隙灯显微镜检查结果。
首次检查时，所有病例均存在角膜或结膜上皮缺损及假膜性结膜炎。类固醇脉冲治疗后皮肤皮疹显著改善。尽管眼部炎症持续数天增加，但假膜消失，角膜和结膜上皮在6周内再生。在慢性期，所有眼睛的角膜均清晰，有Vogt栅栏（POV），提示存在角膜上皮干细胞。所有眼睛的最佳矫正视力均为20/20或更好。5只眼睛出现浅层点状角膜炎。除1只眼睛有轻微穹窿缩短外，无眼睛出现瘢痕性改变。在所有临床过程中未发生类固醇的显著不良反应。
疾病发作时的类固醇脉冲治疗对预防眼部并发症具有重要治疗意义。局部应用倍他米松在预防角膜缘区域角膜上皮干细胞丢失和瘢痕性改变方面也显示出巨大前景。
