Departments of Ophthalmology, Oporto, Portugal.
Ocul Immunol Inflamm. 1993;1(1-2):171-8. doi: 10.3109/09273949309086556.
Toxic epidermal necrolysis (TEN) or Lyell's syndrome is an acute life-threatening disease often triggered by an adverse drug reaction and/or infection. It is characterized by an extensive epidermal loss and severe mucosal involvement. Its pathophysiology is unresolved, but several studies support the hypothesis of an immune mediation. The authors review the ocular involvement of ten drug-induced TEN cases observed in the last four years (eight females and two males; mean age=47). An epidermal loss ranging from 24 to 95% of body surface area was associated with ocular (10/10), oral (9/10), genital (6/10) and anal (4/10) involvement. Ocular findings in the acute phase were: bilateral mucopurulent conjunctivitis (9/10), with inflammatory membranes (3/10), punctate epithelial keratitis (6/10) and corneal ulcers (3/10). Skin palpebral detachment occurred in seven cases, all with extensive and severe systemic disease. In the chronic phase of the disease the authors observed keratoconjunctivitis sicca (4/7), symblepharon (3/7), entropion with trichiasis (2/7) and corneal neovascularization (2/7). HLA B44 (B12) was observed in four of eight studied patients. In conclusion, ocular involvement is frequent in TEN patients and, in those who survive, it significantly contributes to the morbidity of the disease.
中毒性表皮坏死松解症(TEN)或莱尔氏综合征是一种严重的、常由药物不良反应和/或感染引发的、危及生命的急性疾病。其特征为广泛的表皮脱落和严重的黏膜累及。其发病机制尚未明确,但多项研究支持免疫介导的假说。作者回顾了过去四年间观察到的 10 例药物诱导的 TEN 眼部受累病例(8 名女性,2 名男性;平均年龄=47 岁)。这些患者的表皮脱落面积占体表面积的 24%至 95%,眼部(10/10)、口腔(9/10)、生殖器(6/10)和肛门(4/10)均有受累。急性阶段的眼部表现为:双侧脓性黏膜炎(9/10),伴有炎症性膜(3/10)、点状上皮角膜炎(6/10)和角膜溃疡(3/10)。7 例患者出现皮肤睑裂分离,均伴有广泛且严重的系统性疾病。在疾病的慢性阶段,作者观察到干燥性角结膜炎(4/7)、睑球粘连(3/7)、倒睫伴乱睫(2/7)和角膜新生血管化(2/7)。在 8 例研究患者中,有 4 例存在 HLA B44(B12)。总之,TEN 患者眼部受累常见,且在存活的患者中,其显著增加了疾病的发病率。