Linear immunoglobulin A bullous dermatosis

Linear immunoglobulin A bullous dermatosis
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DOI:
10.1016/j.clindermatol.2011.03.008
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发表时间:
2012-01-01
影响因子:
2.7
通讯作者:
Marinkovich, M. Peter
Marinkovich, M. Peter
中科院分区:
医学3区
文献类型:
--
作者:
Fortuna, Giulio;Marinkovich, M. Peter

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线性免疫球蛋白A(伊加)大疱性皮肤病,也称为线性伊加疾病,是一种自身免疫性粘膜皮肤疾病,其特征在于上皮下大疱,具有针对基底膜区中几种不同抗原的伊加自身抗体。其免疫病理学特征在于沿着基底膜区沿着存在连续线性伊加存款,这在直接免疫荧光上清晰可见。成人型和儿童型线性伊加疾病的临床特点和分布不同。诊断是通过临床,组织病理学和免疫病理学检查。两种常见的治疗方法是氨苯砜和磺胺吡啶,它们可以减少炎症反应,并在不同的时间段内实现疾病缓解。(C)12 Elsevier Inc. All rights reserved.
Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time. (C)12 Elsevier Inc. All rights reserved.