大疱性类天疱疮患儿母亲体内抗BP180抗体缺失。
Absence of anti-BP180 antibodies in mothers of infants with bullous pemphigoid.
作者信息
Chiavérini C, Hamel-Teillac D, Gilbert D, Prost Y
机构信息
Department of Dermatology, Hospital Necker-Enfants Malades, Paris, and Clinical and Experimental Immunology Laboratory, University of Medicine, Rouen, France.
出版信息
Br J Dermatol. 2006 May;154(5):839-43. doi: 10.1111/j.1365-2133.2005.07061.x.
BACKGROUND
It is not clear whether bullous pemphigoid (BP) of infancy is linked to maternal transmission of pathogenic autoantibodies. Objectives To search for anti-BP180 antibodies in the sera of infants with BP and their mothers, using sensitive and specific methods.
METHODS
Four infants (<6 months) with BP and their mothers were tested for anti-BP180 antibodies by indirect immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA).
RESULTS
We found anti-BP180 antibodies in the sera of the four infants with all methods. These antibodies reacted with the extracellular domain NC16A. In the serum of their mothers we found 180 and 160 kDa proteins, each in one case, but indirect immunofluorescence and ELISA were negative, suggesting the absence of anti-BP180 autoantibodies reacting with the extracellular domain NC16A.
CONCLUSIONS
BP of infants is not due to maternofetal transmission of pathogenic autoantibodies. Other hypotheses for the pathophysiology of BP are discussed.
背景
婴儿大疱性类天疱疮（BP）是否与致病性自身抗体的母体传播有关尚不清楚。目的：采用灵敏且特异的方法，在患BP的婴儿及其母亲的血清中寻找抗BP180抗体。
方法
对4例年龄小于6个月的患BP的婴儿及其母亲，采用间接免疫荧光法、免疫印迹法和酶联免疫吸附测定（ELISA）检测抗BP180抗体。
结果
我们用所有方法均在4例婴儿血清中发现了抗BP180抗体。这些抗体与细胞外结构域NC16A发生反应。在其母亲的血清中，我们分别在1例中发现了180 kDa和160 kDa的蛋白，但间接免疫荧光法和ELISA均为阴性，提示不存在与细胞外结构域NC16A发生反应的抗BP180自身抗体。
结论
婴儿BP并非由致病性自身抗体的母婴传播所致。文中讨论了BP病理生理学的其他假说。
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