Department of Dermatology, Oxford Radcliffe NHS Trust and University of Oxford, Oxford, UK.
Clin Exp Dermatol. 2010 Jul;35(5):543-5. doi: 10.1111/j.1365-2230.2010.03827.x. Epub 2010 Apr 26.
Lichen sclerosus (LS) is associated with autoimmune disease in female children and adults. In adult women, there are antibody and T-cell responses to proteins in the basement membrane zone (BMZ). The aim of this study was to investigate reactivity to the BMZ in girls with LS. Nine girls with vulval LS were studied clinically and serologically. The presence of circulating BMZ autoantibodies was investigated. Autoimmunity was assessed by personal and family history of autoimmune diseases and autoantibodies. We detected circulating BMZ antibodies in four of the nine children, all with IgG responses. Three patients were positive by indirect immunofluorescence, one had a positive ELISA reaction to bullous pemphigoid antigen (BP)180, and three had a positive reaction on BP180 immunoblots. There was no association with autoimmune disease or clinical features. To our knowledge, this is the first study to find BMZ autoantibodies in children with vulval LS. The autoantibodies were directed at BP180 and were exclusively of the IgG class.
局限性硬皮病（LS）与儿童和成人的自身免疫性疾病有关。在成年女性中，存在针对基底膜带（BMZ）蛋白的抗体和 T 细胞反应。本研究旨在研究患有 LS 的女孩对 BMZ 的反应。对 9 名患有外阴 LS 的女孩进行了临床和血清学研究。研究了循环 BMZ 自身抗体的存在情况。通过自身免疫性疾病和自身抗体的个人和家族史评估自身免疫。我们在 9 名儿童中的 4 名中检测到循环 BMZ 抗体，均为 IgG 反应。3 例患者间接免疫荧光阳性，1 例大疱性类天疱疮抗原（BP）180 ELISA 反应阳性，3 例 BP180 免疫印迹反应阳性。与自身免疫性疾病或临床特征无关。据我们所知，这是第一项在外阴 LS 患儿中发现 BMZ 自身抗体的研究。自身抗体针对 BP180，且均为 IgG 类。
