Epidermolysis bullosa acquisita. Immunofluorescence, electron microscopic and immunoelectron microscopic studies in four patients.
Epidermolysis bullosa acquisita. Immunofluorescence, electron microscopic and immunoelectron microscopic studies in four patients.
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大疱性表皮松解症。
DOI：
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发表时间：
1980
影响因子：
10.3
通讯作者：
G. L. Kalsbeek
中科院分区：
文献类型：
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作者：
C. Nieboer;D. Boorsma;M. Woerdeman;G. L. Kalsbeek
研究了4例获得性大疱性表皮病的临床表现。从皮肤的受累和未受累区域进行活检，并进行免疫组织化学和显微镜下变化研究。直接免疫荧光显示IgG和C3/4沉积在一个线性或norched模式沿着表皮基底膜在参与和未参与的皮肤。此外，还可检出伊加（3/4）、IgM（1/4）、C4（3/4）和备解素（3/4）。间接免疫荧光显示，在一个病人的循环抗体，阿利亚是对上皮基底膜带的存在。常规电子显微镜显示水疱位于真皮中，留下水疱顶部的基底层。用免疫电子显微镜使用过氧化物酶标记的抗体，在体内沉积的IgG被观察到的基底层在真皮的perilisional和未涉及的皮肤。这些观察结果表明，获得性大疱性表皮炎是一个独特的实体，其中自身免疫机制可能发挥作用。
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.