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Abstract：
Linear IgA/IgG bullous dermatosis (LAGBD) is a rare autoimmune subepidermal bullous disorder characterized by linear deposition of concurrent IgA and IgG autoantibodies along the basement membrane zone (BMZ). The clinical features of LAGBD can be diverse, including tense blisters, erosions, erythema, crusting and mucosa involvement, while papules or nodules are generally absent. In this study, we present a unique case of LAGBD, which showed prurigo nodularis-like clinical appearance on physical examination, linear deposition of IgG and C3 along the basement membrane zone (BMZ) in direct immunofluorescence (DIF), IgA autoantibodies against the 97-kDa and 120-kDa of BP180 and IgG autoantibodies against the 97-kDa of BP180 by immunoblotting (IB), while BP180 NC16a domain, BP230, and laminin 332 were negative by enzyme-linked immunosorbent assay (ELISA). After administration of minocycline, the skin lesions improved. We performed a literature review of LAGBD cases with heterogeneous autoantibodies and found clinical presentations of most cases resemble bullous pemphigoid (BP) and linear IgA bullous disease (LABD), which is consistent with previous reported findings. We aim to increase our understanding of this disorder and to enhance the importance of applying immunoblot analyses and other serological detection tools in clinic for precise diagnosis as well as accurate treatment strategy of various autoimmune bullous dermatoses.
摘要：
线性IgA/IgG大疱性皮肤病（LAGBD）是一种罕见的自身免疫性表皮下大疱性疾病，其特征是沿基底膜区（BMZ）并发IgA和IgG自身抗体的线性沉积。LAGBD的临床特征可以是多种多样的，包括紧张的水泡，侵蚀,红斑,结痂和粘膜受累，而丘疹或结节一般不存在。在这项研究中,我们提出了一个独特的LAGBD案例，在体格检查中显示结节性痒疹样临床表现，直接免疫荧光（DIF）中IgG和C3沿基底膜区（BMZ）的线性沉积，通过免疫印迹（IB），针对BP180的97kDa和120kDa的IgA自身抗体和针对BP180的97kDa的IgG自身抗体，而BP180NC16a结构域，通过酶联免疫吸附测定（ELISA），BP230和层粘连蛋白332为阴性。服用米诺环素后，皮肤病变改善。我们对具有异质性自身抗体的LAGBD病例进行了文献综述，发现大多数病例的临床表现类似大疱性类天疱疮（BP）和线性IgA大疱性疾病（LABD）。这与以前报道的发现一致。我们的目标是增加我们对这种疾病的了解，并提高在临床上应用免疫印迹分析和其他血清学检测工具的重要性，以精确诊断以及各种自身免疫性大疱性皮肤病的准确治疗策略。
