弹力纤维染色在营养不良型大疱性表皮松解症中的诊断价值

目的:通过免疫组织化学染色回顾性诊断营养不良型大疱性表皮松解症(DEB)患者,并了解弹力纤维在DEB和大疱性类天疱疮(BP)患者皮损中的分布情况,进一步探讨弹力纤维染色在DEB诊断中的临床意义。方法:收集17例DEB和3例BP患者皮损标本和临床资料,采用免疫组织化学染色方法检测角蛋白14、层粘蛋白5和胶原蛋白Ⅳ、Ⅶ在DEB患者中的表达,弹力纤维染色检测弹力纤维在DEB患者和BP患者皮损中的分布情况。结果:17例DEB患者皮损中,角蛋白14和层粘蛋白5在真、表皮裂隙的表皮侧表达,胶原蛋白Ⅳ在表皮侧表达或表达缺失,而胶原蛋白Ⅶ表达缺失。弹力纤维在17例DEB患者真皮浅中层均缺失,而在BP患者真皮乳头处减少。结论:DEB可根据角蛋白14、层粘蛋白5、胶原蛋白Ⅳ和胶原蛋白Ⅶ在皮损中的免疫组化检测结果诊断。弹力纤维在DEB和BP患者皮肤中分布不同,弹力纤维染色可作为DEB和BP的鉴别诊断依据之一。

Diagnostic value of elastic fibers staining in dystrophic epidermolysis bullosa

Objective：To investigate the diagnostic value of elastic fibers staining in dystrophic epidermolysis bullosa (DEB) that was diagnosed based on immunohistochemistry staining.Methods:Immunohistochemistry staining was used to examine keratin 14, laminin 5, collagen Ⅳ and collagen Ⅶ in DEB, and Weigert’s resorcin fuchsin staining for elastic fibers was used in DEB and BP. Results: As for 17 cases with DEB, collagen Ⅶ was absent at the dermis epidermis junction, while keratin 14 and laminin 5 were present at the epidermal side and collagen Ⅳ distributed at the epidermal side or was absent. Elastic fibers were completely absent in papillary and upper dermis in DEB while were reduced only in dermal papillae in BP. Conclusions：DEB can be diagnosed based on the immunohistochemistry staining results of keratin 14, laminin 5, collagen Ⅳ and collagen Ⅶ.According to the different expression patterns of elastic fibers in DEB and BP, elastic fibers staining could be an alternative marker to differentiate them.