Michalaki H, Staquet M J, Cerri A, Berti E, Roche P, Machado P, Nicolas J F
INSERM U. 346, Department of Dermatology, Claude Bernard University, Lyon, France.
J Invest Dermatol. 1992 Feb;98(2):204-8. doi: 10.1111/1523-1747.ep12555875.
The integrin alpha 6 beta 4 complex is a protein of the membrane of basal keratinocytes, localized at the surface of cells in contact with the basement membrane zone in normal skin. The expression of alpha 6 beta 4 was investigated in several autoimmune blistering skin diseases including bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), bullous systemic lupus erythematosus (BSLE), and pemphigus vulgaris (PV) by an indirect immunofluorescence technique. In lesional bullous skin of BP, alpha 6 beta 4 expression was either absent, or in some cases represented an unusual irregular patchy staining. In contrast, in lesional bullous skin from EBA, BSLE, and PV, alpha 6 beta 4 expression was comparable to that observed in normal skin, i.e., a linear staining of the BMZ. Thus, analysis of the alpha 6 beta 4 integrin reactivity on lesional skin, in conjunction with the typical localization of collagen IV, allows a rapid and accurate distinction between BP and EBA.
整合素α6β4复合物是基底角质形成细胞细胞膜上的一种蛋白质，位于正常皮肤中与基底膜带接触的细胞表面。采用间接免疫荧光技术，对包括大疱性类天疱疮（BP）、获得性大疱性表皮松解症（EBA）、大疱性系统性红斑狼疮（BSLE）和寻常型天疱疮（PV）在内的几种自身免疫性水疱性皮肤病中α6β4的表达进行了研究。在BP的皮损水疱皮肤中，α6β4表达缺失，或在某些情况下表现为异常的不规则斑片状染色。相比之下，在EBA、BSLE和PV的皮损水疱皮肤中，α6β4表达与正常皮肤中观察到的情况相当，即基底膜带呈线性染色。因此，结合Ⅳ型胶原的典型定位，分析皮损皮肤中α6β4整合素的反应性，可快速准确地区分BP和EBA。
