Smith J R, Kupa A, Coster D J
Department of Ophthalmology, Flinders University of South Australia and Flinders Medical Centre, Bedford Park, South Australia, Australia.
Aust N Z J Ophthalmol. 1999 Dec;27(6):443-6. doi: 10.1046/j.1440-1606.1999.00266.x.
A case of linear IgA disease is reported to alert ophthalmologists and physicians to this unusual cause of chronic cicatrizing conjunctivitis.
Clinical records of a patient suffering from linear IgA disease were reviewed.
A 65-year-old woman with a complicated medical history experienced rapidly progressive chronic cicatrizing conjunctivitis leading to corneal perforation. Undiagnosed gingivitis and palatal ulceration had been present for 5 years prior to the onset of ocular symptoms and vitamin C deficiency had followed the consequent dietary restrictions. A diagnosis of linear IgA disease was made on conjunctival biopsy, which demonstrated linear deposits of IgA along the epithelial basement membrane. The perforation was managed successfully with a conjunctival pediculate flap. Control of the inflammation was achieved with systemic prednisolone and cyclophosphamide but at the expense of serious systemic side-effects.
Linear IgA disease causes progressive conjunctival cicatrization in many affected individuals. Although dapsone generally controls the inflammation, heavier systemic immunosuppression was required in this case. Involvement of skin or other mucosal surfaces may become symptomatic before the conjunctivitis, and physicians must be educated to refer patients for ophthalmological review on diagnosis. Conversely, ophthalmologists encountering ocular linear IgA disease should be aware of the possibility of other mucosal involvement requiring physician intervention.
报告一例线状IgA疾病病例，以提醒眼科医生和内科医生注意这种导致慢性瘢痕性结膜炎的不寻常病因。
回顾了一名患有线状IgA疾病患者的临床记录。
一名有复杂病史的65岁女性经历了迅速进展的慢性瘢痕性结膜炎，导致角膜穿孔。在眼部症状出现前5年就存在未被诊断的牙龈炎和腭部溃疡，随后因饮食限制出现维生素C缺乏。结膜活检诊断为线状IgA疾病，显示IgA沿上皮基底膜呈线状沉积。穿孔通过结膜带蒂瓣成功处理。全身应用泼尼松龙和环磷酰胺控制了炎症，但付出了严重全身副作用的代价。
线状IgA疾病在许多受累个体中导致进行性结膜瘢痕形成。虽然氨苯砜通常能控制炎症，但该病例需要更强的全身免疫抑制治疗。皮肤或其他黏膜表面受累可能在结膜炎之前出现症状，必须教育内科医生在诊断时将患者转诊至眼科进行检查。相反，遇到眼部线状IgA疾病的眼科医生应意识到可能存在需要内科医生干预的其他黏膜受累情况。
