Bolte C, Gonzalez S
Department of Pathology, School of Medicine, Catholic University of Chile, Santiago, Chile.
Am J Dermatopathol. 1995 Dec;17(6):580-3. doi: 10.1097/00000372-199512000-00009.
A simple and rapid procedure to diagnose major variants of congenital epidermolysis bullosa (EB) by means of immunohistochemical staining of collagen IV (CIV) in formalin-fixed and paraffin-embedded samples is described. Sixteen cases were stained with a monoclonal antibody (clone CIV 22) against collagen IV present in the lamina densa layer of the basement membrane. Electron microscopy was performed simultaneously. Twelve cases showed one of two staining patterns: an EB simplex pattern (seven cases) consisting of linear and continuous staining on the "floor" of the blister and an EB dystrophica pattern (five cases) showing a linear and discontinuous staining on the "roof" of the blister. In four cases, collagen IV staining was not reactive because of irreversible antigen loss due to inadequate fixation and/or embedding. In those cases with positive staining, an excellent correlation with electron microscopic diagnosis was achieved. This procedure allows a rapid diagnosis of major EB variants, especially where electron microscopy is not available, and also facilitates diagnosis by optimally orienting specimens for ultrastructural examination when available.
本文描述了一种通过对福尔马林固定石蜡包埋样本中的IV型胶原（CIV）进行免疫组织化学染色来诊断先天性大疱性表皮松解症（EB）主要变异型的简单快速方法。16例样本用针对基底膜致密层中IV型胶原的单克隆抗体（克隆CIV 22）进行染色。同时进行电子显微镜检查。12例呈现两种染色模式之一：单纯性EB模式（7例），表现为水疱“底部”呈线性连续染色；营养不良性EB模式（5例），表现为水疱“顶部”呈线性间断染色。4例由于固定和/或包埋不充分导致不可逆的抗原丢失，IV型胶原染色无反应。在那些染色阳性的病例中，与电子显微镜诊断结果具有极好的相关性。该方法能够快速诊断主要的EB变异型，特别是在没有电子显微镜的情况下，并且在有电子显微镜时，通过最佳地定向标本进行超微结构检查也有助于诊断。
