Nieboer C, Boorsma D M, Woerdeman M J, Kalsbeek G L
Br J Dermatol. 1980 Apr;102(4):383-92. doi: 10.1111/j.1365-2133.1980.tb06550.x.
Four patients with the clinical picture of epidermolysis bullosa acquisita were investigated. Biopsies were taken from the involved and uninvolved areas of the skin and the immunohistochemical and microscopic changes were studied. Direct immunofluorescence showed deposition of IgG and C3/4 in a linear or norched pattern along the epidermal basement membrane in both the involved and the uninvolved skin. In addition IgA (3/4), IgM (1/4), C4 (3/4) and properdin (3/4) could be detected. Indirect immunofluorescence revealed the presence of circulating antibodies against inter alia the epithelial basement membrane zone in one patient. Routine electron microscopy showed that the blister was situated in the dermis leaving the basal lamina in the roof of the blister. With immunoelectron microscopy using peroxidase-labelled antibody the in vivo deposition of IgG was observed just beneath the basal lamina in the dermis of both the perilesional and the uninvolved skin. These observations show that epidermolysis bullosa acquisita is a distinct entity, in which autoimmune mechanisms might possibly play a role.
对4例获得性大疱性表皮松解症临床表现的患者进行了研究。从皮肤的受累及未受累区域取活检组织，研究免疫组化和显微镜下的变化。直接免疫荧光显示，在受累皮肤和未受累皮肤中，IgG和C3/4沿表皮基底膜呈线性或锯齿状沉积。此外，还可检测到IgA（3/4）、IgM（1/4）、C4（3/4）和备解素（3/4）。间接免疫荧光显示，1例患者存在针对上皮基底膜区等的循环抗体。常规电子显微镜检查显示，水疱位于真皮内，水疱顶部留有基膜。使用过氧化物酶标记抗体的免疫电子显微镜检查发现，在病损周围皮肤和未受累皮肤的真皮中，IgG在基膜下方有体内沉积。这些观察结果表明，获得性大疱性表皮松解症是一种独特的疾病实体，其中自身免疫机制可能起作用。
