Trüeb R M, Didierjean L, Fellas A, Elias A, Borradori L
Department of Dermatology, University Hospital of Zurich, Switzerland.
J Am Acad Dermatol. 1999 Feb;40(2 Pt 2):338-44. doi: 10.1016/s0190-9622(99)70481-0.
The clinical and immunopathologic features of children with acquired subepidermal blistering disorders show considerable overlap, and their classification frequently requires characterization of the targeted antigens. A 8-month-old boy developed a generalized subepidermal blistering disorder with striking palmoplantar involvement. The patient's serum contained antibodies reacting against the epidermal side of 1 M sodium chloride separated normal human skin. Immunoblotting analysis demonstrated circulating IgG autoantibodies that reacted against a eukaryotic recombinant form of human bullous pemphigoid antigen 180 (BP180). In addition, the patient had circulating IgG autoantibodies that bound a protein of 120 kDa in skin basement membrane zone extracts, that might correspond to the linear IgA bullous disease (LABD) antigen. This study illustrates that a child with clinical and immunopathologic features considered characteristic of childhood bullous pemphigoid (BP) had circulating IgG antibodies that bound to an eukaryotic recombinant form of human BP180, and hence, fulfilled the diagnostic criteria of BP. Review of the literature disclosed only 10 cases of childhood BP, that were characterized on the basis of the targeted antigens. The concomitant presence of circulating IgG autoantibodies against BP180 and a 120 kDa protein may signify either coexistence of autoantibodies with distinct specificities or reflect antigenic cross-reactivity between BP180 and the 120/97 LABD antigen.
获得性表皮下大疱性疾病患儿的临床和免疫病理特征有相当大的重叠，其分类常常需要对靶抗原进行特征描述。一名8个月大的男童患上了一种全身性表皮下大疱性疾病，掌跖部受累明显。患者血清中含有与1M氯化钠分离的正常人皮肤表皮侧发生反应的抗体。免疫印迹分析显示循环IgG自身抗体与人类大疱性类天疱疮抗原180（BP180）的真核重组形式发生反应。此外，患者还具有循环IgG自身抗体，其在皮肤基底膜带提取物中与一种120kDa的蛋白质结合，该蛋白质可能对应于线状IgA大疱性疾病（LABD）抗原。本研究表明，一名具有被认为是儿童大疱性类天疱疮（BP）特征性临床和免疫病理特征的儿童，其循环IgG抗体与人类BP180的真核重组形式结合，因此符合BP的诊断标准。文献回顾仅发现10例儿童BP病例，这些病例是根据靶抗原进行特征描述的。针对BP180和一种120kDa蛋白质的循环IgG自身抗体同时存在，可能意味着具有不同特异性的自身抗体共存，或者反映了BP180与120/97 LABD抗原之间的抗原交叉反应。
