Prieto V G, McNutt N S
Department of Pathology, New York Hospital Cornell Univ. Medical Center, 10021.
J Cutan Pathol. 1994 Apr;21(2):118-22. doi: 10.1111/j.1600-0560.1994.tb00245.x.
Epidermolysis bullosa simplex (EBS) is an uncommon genetic skin disease characterized by fragility of the basal keratinocytes and propensity to develop blisters. While a panel of antibodies against type IV collagen, laminin, and bullous pemphigoid antigen has been used to identify cases of EBS in frozen sections, we have found that a monoclonal antibody detecting cytokeratin in basal keratinocytes is useful in paraffin sections. Formalin-fixed, paraffin-embedded tissues from 12 patients with EBS were studied with the following monoclonal antibodies: MNF116 (DAKO, Carpinteria, CA), CAM 5.2 (Becton Dickinson, San Jose, CA), and AE1-AE3 mixture (Boehringer Mannheim Corp, Indianapolis, IN). Histologically, all the cases had focal vacuolization of the basal cell layer, with areas of dermal-epidermal separation. MNF116 was strongly positive in the basal keratinocytes, including the vacuolated ones, and demonstrated the presence of fragments of keratinocytes attached to the floor of the blister. CAM 5.2 stained sweat ducts only. AE1-AE3 was weakly positive in the basal cells, and almost completely negative on the fragmented basal cell keratinocytes. We consider that the immunostain with MNF116 in tissues fixed routinely in formalin and embedded in paraffin is helpful for the direct demonstration of the level of splitting in EBS.
单纯性大疱性表皮松解症（EBS）是一种罕见的遗传性皮肤病，其特征为基底角质形成细胞脆弱且易于形成水疱。虽然一组针对IV型胶原、层粘连蛋白和大疱性类天疱疮抗原的抗体已被用于在冰冻切片中识别EBS病例，但我们发现一种检测基底角质形成细胞中细胞角蛋白的单克隆抗体在石蜡切片中很有用。我们用以下单克隆抗体研究了12例EBS患者经福尔马林固定、石蜡包埋的组织：MNF116（DAKO，卡平特里亚，加利福尼亚州）、CAM 5.2（贝克顿·迪金森公司，圣何塞，加利福尼亚州）和AE1 - AE3混合物（勃林格殷格翰公司，印第安纳波利斯，印第安纳州）。组织学上，所有病例的基底细胞层均有局灶性空泡化，并伴有真皮 - 表皮分离区域。MNF116在基底角质形成细胞中呈强阳性，包括空泡化的细胞，并显示有附着在水疱底部的角质形成细胞碎片。CAM 5.2仅对汗腺导管染色。AE1 - AE3在基底细胞中呈弱阳性，在破碎的基底细胞角质形成细胞上几乎完全阴性。我们认为，在常规用福尔马林固定并石蜡包埋的组织中用MNF116进行免疫染色有助于直接显示EBS中的分裂水平。
