Burge S M, Garrod D R
Department of Dermatology, Slade Hospital, Oxford, U.K.
Br J Dermatol. 1991 Mar;124(3):242-51. doi: 10.1111/j.1365-2133.1991.tb00568.x.
The immunocytochemical distribution of desmosomal components was determined in involved skin from eight patients with Darier's disease, five patients with Hailey-Hailey disease and two patients with transient acantholytic dermatosis as well as skin from four normal controls. Sections were stained using monoclonal antibodies to the desmosomal proteins dp1 and dp2 (desmoplakins) and the desmosomal glycoproteins dg1 (desmoglein), and dg2 and dg3 (desmocollins). There was normal expression of desmosomal proteins and glycoproteins at the periphery of the keratinocytes in the perilesional skin in Darier's disease, in Hailey-Hailey disease and in transient acantholytic dermatosis. In the lesional skin there was reduced expression of desmosomal proteins and glycoproteins in the basaloid 'buds' at the base of the lesions, but there was bright diffuse staining of the acantholytic cells. Focal intracellular staining was detected within many of the acantholytic keratinocytes in Hailey-Hailey disease and within some of these cells in Darier's disease. Suction blisters were used to induce fresh acantholysis in lesional skin in Darier's disease and clinically uninvolved skin in Hailey-Hailey disease. The results indicated that acantholysis precedes the development of intracellular staining. Although there are immunopathological abnormalities in the distribution of desmosomal proteins and glycoproteins in both Darier's disease and Hailey-Hailey disease, the changes are probably secondary to internalization of desmosomal components with breakdown and redistribution of antigens rather than a primary deficiency in the synthesis of these proteins. Focal internalization was more widespread in Hailey-Hailey disease than in Darier's disease and the differences in the distribution of desmosomal components in these diseases confirm that they are distinct entities.
研究测定了8例 Darier病患者、5例Hailey-Hailey病患者和2例暂时性棘层松解性皮病患者受累皮肤以及4例正常对照者皮肤中桥粒成分的免疫细胞化学分布。切片用针对桥粒蛋白dp1和dp2（桥粒斑蛋白）以及桥粒糖蛋白dg1（桥粒芯糖蛋白）、dg2和dg3（桥粒胶蛋白）的单克隆抗体进行染色。在Darier病、Hailey-Hailey病和暂时性棘层松解性皮病的损害周围皮肤中，角质形成细胞周边的桥粒蛋白和糖蛋白表达正常。在损害皮肤中，损害底部的基底样“芽”内桥粒蛋白和糖蛋白表达减少，但棘层松解细胞有明亮的弥漫性染色。在Hailey-Hailey病的许多棘层松解角质形成细胞内以及Darier病的部分此类细胞内检测到局灶性细胞内染色。在Darier病的损害皮肤和Hailey-Hailey病临床上未受累的皮肤中，用吸引水疱诱导新鲜的棘层松解。结果表明棘层松解先于细胞内染色的出现。尽管Darier病和Hailey-Hailey病在桥粒蛋白和糖蛋白分布方面均存在免疫病理异常，但这些变化可能继发于桥粒成分的内化以及抗原的分解和重新分布，而非这些蛋白质合成的原发性缺陷。与Darier病相比，局灶性内化在Hailey-Hailey病中更为普遍，这些疾病中桥粒成分分布的差异证实它们是不同的疾病实体。
