Holsclaw D S
Francis I. Proctor Foundation, Department of Ophthalmology, University of California, San Fransisco 94143, USA.
Int Ophthalmol Clin. 1998 Fall;38(4):89-106. doi: 10.1097/00004397-199803840-00009.
CP is a systemic disease of presumed autoimmune etiology that may involve the skin, conjunctiva, or any mucosal surface. As many patients present with ocular symptoms, ophthalmologists must inquire about signs of potential life-threatening esophageal and laryngeal involvement. Ocular involvement is a bilateral chronic cicatricial conjunctivitis that may result in fornix foreshortening, symblepharon formation, trichiasis, and entropion and keratinization of the eyelids and conjunctiva. These mechanical factors are responsible for progressive scarring and vascularization of the ocular surface. Successful systemic treatment of the autoimmune disease is now available; however, the condition often is not diagnosed until significant structural damage to the ocular adnexa has occurred. Our ability to rehabilitate visually patients with advanced disease by penetrating keratoplasty and associated techniques remains extremely limited. Hence, the focus of treatment in OCP should be on early detection of the disease, including conjunctival biopsy, prompt systemic treatment to prevent disease progression, and early aggressive treatment of secondary eyelid and conjunctival abnormalities to avoid the blinding keratopathy so prevalent in this disease.
瘢痕性类天疱疮是一种病因推测为自身免疫性的全身性疾病，可累及皮肤、结膜或任何黏膜表面。由于许多患者表现出眼部症状，眼科医生必须询问是否有潜在危及生命的食管和喉部受累迹象。眼部受累表现为双侧慢性瘢痕性结膜炎，可导致穹窿缩短、睑球粘连形成、倒睫、睑内翻以及眼睑和结膜角化。这些机械因素导致眼表进行性瘢痕形成和血管化。目前已有针对这种自身免疫性疾病的成功全身治疗方法；然而，在眼附属器发生明显结构损伤之前，该病往往无法得到诊断。我们通过穿透性角膜移植术及相关技术使晚期疾病患者恢复视力的能力仍然极为有限。因此，瘢痕性类天疱疮的治疗重点应是疾病的早期检测，包括结膜活检，及时进行全身治疗以防止疾病进展，以及早期积极治疗继发性眼睑和结膜异常，以避免该病中普遍存在的致盲性角膜病变。
