Hoang-Xuan T, Robin H, Heller M, Caux F, Prost C
Department of Ophthalmology, Hospital Bichat, Paris, France.
Ophthalmology. 1997 Sep;104(9):1414-20. doi: 10.1016/s0161-6420(97)30122-5.
To describe for the first time the direct immunoelectron microscopic pattern of immune deposits on the conjunctival basement membrane in epidermolysis bullosa acquisita (EBA).
Case reports.
Two patients.
Epidermolysis bullosa acquisita associated with cicatrizing conjunctivitis.
Direct immunofluorescence and direct immunoelectron microscopy without freezing on conjunctival and skin biopsy specimens, indirect immunofluorescence, Western immunoblot analysis.
Results of direct immunoelectron microscopic examination of the conjunctiva showed the presence of immune deposits in the anchoring fibril zone, just beneath the lamina densa, in both patients. This finding was the same as the direct immunoelectron microscopic pattern shown in the skin of these patients, which is known to be very specific for EBA. Direct immunofluorescence was positive in the conjunctiva of only one patient. Indirect immunofluorescence and Western immunoblot analysis failed to detect circulating autoantibodies.
Direct immunoelectron microscopy on the conjunctiva is a useful diagnostic tool to differentiate EBA from other related autoimmune mucocutaneous blistering diseases.
首次描述获得性大疱性表皮松解症（EBA）患者结膜基底膜上免疫沉积物的直接免疫电子显微镜模式。
病例报告。
两名患者。
与瘢痕性结膜炎相关的获得性大疱性表皮松解症。
对结膜和皮肤活检标本进行非冷冻的直接免疫荧光和直接免疫电子显微镜检查、间接免疫荧光、蛋白质免疫印迹分析。
两名患者结膜的直接免疫电子显微镜检查结果均显示，在致密板下方的锚原纤维区存在免疫沉积物。这一发现与这些患者皮肤的直接免疫电子显微镜模式相同，而后者已知对EBA具有高度特异性。仅一名患者的结膜直接免疫荧光呈阳性。间接免疫荧光和蛋白质免疫印迹分析未能检测到循环自身抗体。
结膜直接免疫电子显微镜检查是将EBA与其他相关自身免疫性黏膜皮肤水疱病相鉴别的有用诊断工具。
