IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature.
IgA-mediated epidermolysis bullosa acquisita: two cases and review of the literature.
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IgA介导的大疱性表皮松解症：两例并文献复习。
DOI：
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发表时间：
2002
期刊：
影响因子：
--
通讯作者：
M. Jonkman
中科院分区：
文献类型：
--
作者：
R. Vodegel;M. D. de Jong;H. Pas;M. Jonkman
我们报告2例成人表皮下大疱性皮肤病，在直接免疫电子显微镜下发现沿着表皮基底膜区线状IgA沉积，沉积在致密层下区。对患者血清的间接免疫荧光显微镜显示，循环中的IgA自身抗体仅与盐裂皮肤基质的真皮部位结合。用真皮和角质形成细胞提取液进行免疫印迹分析均为阴性。使用III型胶原缺陷性皮肤的间接免疫荧光显微镜(“敲除”底物)显示没有IgA结合，而在正常人皮肤的表皮基底膜区域可见线性的IgA结合。因此，患者体内的自身抗原是III型胶原。诊断为获得性大疱性表皮松解症(IgA-EBA)。我们根据以下标准系统地回顾了这一亚型线状IgA皮肤病患者的文献：间接或直接免疫电子显微镜显示血清-IgA与盐裂皮肤真皮侧或致密带下的IgA沉积具有排他性。我们了解到，IgA-EBA在临床上与经典的“板层-透光型”线状IgA皮肤病或免疫球蛋白介导的获得性大疱性表皮松解症(Ig G-EBA)无法区分。只有少数的IgA-EBA患者表现出糜烂或疤痕，或有像机械性空洞型Ig G-EBA那样的抗药性眼部症状。大多数IgA-EBA患者对氨苯砜治疗有反应。
We describe 2 adult patients with a subepidermal bullous dermatosis with exclusively linear IgA depositions along the epidermal basement membrane zone that were deposited in the sublamina densa zone as witnessed by direct immunoelectron microscopy. Indirect immunofluorescence microscopy of patients' sera revealed circulating IgA autoantibodies that bound exclusively to the dermal site of salt-split skin substrate. Immunoblot analysis using dermal and keratinocyte extracts were negative. Indirect immunofluorescence microscopy using type VII collagen-deficient skin ("knockout" substrate) showed no IgA binding, whereas linear IgA binding was seen at the epidermal basement membrane zone in normal human skin. The autoantigen in the patients was thus type VII collagen. A diagnosis of IgA-mediated epidermolysis bullosa acquisita (IgA-EBA) was made. We systematically reviewed the literature of this subset of patients with linear IgA dermatosis on the basis of the following criteria: exclusive binding of serum-IgA to the dermal side of salt-split skin or IgA depositions in the sublamina densa zone by indirect or direct immunoelectron microscopy. We learned that IgA-EBA is clinically indistinguishable from the classic "lamina-lucida type" linear IgA dermatosis or from the inflammatory type of IgG-mediated epidermolysis bullosa acquisita (IgG-EBA). Only a minority of the patients with IgA-EBA showed milia or scarring or had therapy-resistant ocular symptoms as in the mechanobullous type of IgG-EBA. Most patients with IgA-EBA responded to dapsone therapy.
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大疱性表皮松解症中皮肤基底膜自身抗原的鉴定。
DOI：
10.1056/nejm198404193101602
发表时间：
1984
期刊：
The New England journal of medicine
影响因子：
--
作者：
Woodley,DT;Briggaman,RA;O'Keefe,EJ;Inman,AO;Queen,LL;Gammon,WR
通讯作者：
Gammon,WR
97 kDa 线性 IgA 大疱性疾病抗原与 180 kDa 大疱性类天疱疮抗原 BPAg2 的部分胞外结构域相同。
DOI：
10.1046/j.1523-1747.1998.00129.x
发表时间：
1998
期刊：
The Journal of investigative dermatology
影响因子：
--
作者：
Zone,JJ;Taylor,TB;Meyer,LJ;Petersen,MJ
通讯作者：
Petersen,MJ
影响因子：
6.5
作者：
Chen, M;Chan, LS;Woodley, DT
通讯作者：
Woodley, DT
线性 IgA 疾病的免疫印迹研究。
影响因子：
4.6
作者：
Dmochowski,M;Hashimoto,T;Bhogal,BS;Black,MM;Zone,JJ;Nishikawa,T
通讯作者：
Nishikawa,T
鉴定人 VII 型胶原上单克隆抗体 LH 7.2 和克隆 I, 185 的表位。
影响因子：
4.6
作者：
Lapiere,JC;Hu,L;Iwasaki,T;Chan,LS;Peavey,C;Woodley,DT
通讯作者：
Woodley,DT