Kraft J, Lieb W, Zeitler P, Schuster V
Department of Ophthalmology, Julius Maximilian University, Würzburg, Germany.
Graefes Arch Clin Exp Ophthalmol. 2000 Sep;238(9):797-800. doi: 10.1007/s004170000172.
Ligneous conjunctivitis is a rare form of chronic recurrent pseudomembranous disease and may be associated with systemic membranous pathological changes. Recently ligneous conjunctivitis has been linked to severe type I plasminogen deficiency. We report on a patient with plasminogen deficiency and severe bilateral ligneous conjunctivitis. A new treatment approach and its outcome in this patient are described.
We present the case of a 9-month-old Turkish girl with massive swelling of the eyelids and hard white pseudomembranes on both lids. The conjunctival smear was positive for Streptococcus pneumoniae. The clinical diagnosis was: ligneous conjunctivitis with superinfection. Histological investigation showed fibrin as major component of the pseudomembranes. The coagulation analyses revealed decreased plasminogen activity (<5%; normal 80-120%) and decreased plasminogen antigen (<0.4 mg/dl; normal 6-25 mg/dl). The failure of surgical therapy led to the attempt at treatment with intravenous lys-plasminogen. A significant improvement of the ocular symptoms occurred; stabilization with no recurrent pseudomembranes could be achieved for 6 months after treatment.
The initial amelioration of symptoms in our patient after systemic replacement therapy confirms the etiological importance of plasminogen deficiency in the development of ligneous conjunctivitis. Curative treatment of ligneous conjunctivitis is still not available. However, intravenous application of plasminogen offers new possibilities in therapy, although long-term treatment seems necessary.
木质化性结膜炎是一种罕见的慢性复发性假膜性疾病，可能与全身膜性病理改变有关。最近，木质化性结膜炎已与严重的I型纤溶酶原缺乏症相关联。我们报告了一名患有纤溶酶原缺乏症和严重双侧木质化性结膜炎的患者。描述了该患者的一种新治疗方法及其结果。
我们介绍了一名9个月大的土耳其女孩的病例，她的眼睑出现大量肿胀，双侧眼睑有坚硬的白色假膜。结膜涂片肺炎链球菌呈阳性。临床诊断为：伴有二重感染的木质化性结膜炎。组织学检查显示纤维蛋白是假膜的主要成分。凝血分析显示纤溶酶原活性降低（<5%；正常80 - 120%），纤溶酶原抗原降低（<0.4mg/dl；正常6 - 25mg/dl）。手术治疗失败后尝试用静脉注射赖氨酸纤溶酶原进行治疗。眼部症状有显著改善；治疗后6个月可实现病情稳定，无假膜复发。
我们的患者在全身替代治疗后症状最初得到改善，这证实了纤溶酶原缺乏在木质化性结膜炎发病机制中的病因学重要性。木质化性结膜炎的根治性治疗仍然没有。然而，静脉注射纤溶酶原提供了新的治疗可能性，尽管似乎需要长期治疗。
