Chimanovitch I, Hamm H, Georgi M, Kroiss M, Stolz W, Apitz C, Bröcker E B, Zillikens D
Department of Dermatology, University of Würzburg, Germany.
Arch Dermatol. 2000 Apr;136(4):527-32. doi: 10.1001/archderm.136.4.527.
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease of the elderly that rarely occurs in children. Most adult BP serum samples react with epitopes within the NC16A domain of BP180, a glycoprotein of the cutaneous basement membrane zone.
To characterize the autoimmune response in childhood BP using recombinant forms of BP180 and to determine the subclass distribution of autoantibodies and their correlation with disease activity.
Serum samples from 2 infants with BP, aged 4 and 5 months, reacted by immunoblot analysis with 4 epitopes clustered within the N-terminal 45 amino acids of the NC16A domain. The same 4 epitopes have previously been shown to be the target in adult BP. Childhood BP antibodies to BP180 NC16A belonged to IgG1, IgG2, IgG3, and IgG4 immunoglobulin subclasses. IgE reactivity was not detected. Serum levels of antibodies targeting BP180 NC16A paralleled disease activity as detected by enzyme-linked immunosorbent assay.
The fine specificity of autoantibodies to BP180 is the same in BP of childhood and adulthood. Childhood BP is a true variant of BP.
大疱性类天疱疮（BP）是一种老年人的自身免疫性表皮下大疱性疾病，儿童罕见。大多数成人BP血清样本与皮肤基底膜区糖蛋白BP180的NC16A结构域内的表位发生反应。
使用BP180重组形式来表征儿童BP中的自身免疫反应，并确定自身抗体的亚类分布及其与疾病活动的相关性。
2例年龄分别为4个月和5个月的BP婴儿的血清样本，通过免疫印迹分析与聚集在NC16A结构域N端45个氨基酸内的4个表位发生反应。先前已证明相同的4个表位是成人BP的靶点。儿童BP针对BP180 NC16A的抗体属于IgG1、IgG2、IgG3和IgG4免疫球蛋白亚类。未检测到IgE反应性。通过酶联免疫吸附测定法检测，靶向BP180 NC16A的抗体血清水平与疾病活动平行。
儿童和成人BP中针对BP180的自身抗体的精细特异性相同。儿童BP是BP的一种真正变体。
