Schofield O M, Fine J D, Verrando P, Heagerty A H, Ortonne J P, Eady R A
Department of Cell Pathology, St. Thomas' Hospital, London, U.K.
J Am Acad Dermatol. 1990 Dec;23(6 Pt 1):1078-83. doi: 10.1016/0190-9622(90)70336-g.
GB3 monoclonal antibody detects a normal basement membrane component (GB3 antigen) that is variably expressed in junctional epidermolysis bullosa. To assess the accuracy of GB3 in the diagnosis of junctional epidermolysis bullosa, we have reviewed its use in 250 cases of the major types of epidermolysis bullosa. In the majority of cases of the simplex and dystrophic forms of epidermolysis bullosa, GB3 antigen is normally expressed. In the Herlitz variant of junctional epidermolysis bullosa, GB3 antigen expression is consistently abnormal, but in the non-Herlitz and indeterminate forms of junctional epidermolysis bullosa, 40% of cases express GB3 antigen normally. We propose that GB3 monoclonal antibody is useful in the accurate identification of patients with Herlitz junctional epidermolysis bullosa and may prove equal to electron microscopy for the diagnosis of this disease. For the non-Herlitz variants, it should not be used as an alternative to electron microscopy but may be of special value in the determination of prognosis.
GB3单克隆抗体可检测到一种正常的基底膜成分（GB3抗原），该成分在交界性大疱性表皮松解症中表达各异。为评估GB3在诊断交界性大疱性表皮松解症中的准确性，我们回顾了其在250例主要类型大疱性表皮松解症中的应用情况。在大多数单纯型和营养不良型大疱性表皮松解症病例中，GB3抗原表达正常。在交界性大疱性表皮松解症的赫利茨变异型中，GB3抗原表达始终异常，但在交界性大疱性表皮松解症的非赫利茨型和不确定型中，40%的病例GB3抗原表达正常。我们认为GBx单克隆抗体有助于准确识别赫利茨交界性大疱性表皮松解症患者，在诊断该疾病方面可能与电子显微镜效果相当。对于非赫利茨变异型，它不应作为电子显微镜的替代方法，但在判断预后方面可能具有特殊价值。
