Han Jung Hyun, Yun Sook Jung, Kim Seong-Jin, Lee Seung-Chul, Won Young Ho, Lee Jee-Bum
Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea.
Ann Dermatol. 2011 May;23(2):209-12. doi: 10.5021/ad.2011.23.2.209. Epub 2011 May 27.
Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.
儿童慢性大疱性疾病（CBDC）是一种自身免疫性水疱病，其特征是免疫球蛋白A（IgA）沉积于基底膜带。CBDC患者血清中的IgA自身抗体（aAbs）与97 kDa（LABD97）和120 kDa（LAD-1）的抗原发生反应，这两种抗原均为大疱性类天疱疮180（BP180，XVII型胶原）细胞外结构域的片段。CBDC血清与BP180的免疫显性NC16a结构域发生反应，该结构域是大疱性类天疱疮患者中IgG aAbs识别的主要区域。一名5岁男孩脐部和腹股沟区出现多发瘙痒性紧张水疱6周。皮损周围区域的直接免疫荧光显示基底膜带处有IgA线性沉积。通过免疫印迹和酶联免疫吸附测定（ELISA），我们鉴定出与分子量为120 kDa的抗原（LAD-1）反应的IgA aAbs，LAD-1是BP180细胞外结构域的一个片段。
