Case of autoimmune intraepidermal and subepidermal blistering disease in which autoantibodies to desmoglein 1 and BP230 coexist
Case of autoimmune intraepidermal and subepidermal blistering disease in which autoantibodies to desmoglein 1 and BP230 coexist
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桥粒芯糖蛋白 1 和 BP230 自身抗体共存的自身免疫性表皮内和表皮下水疱病病例
DOI：
10.1111/1346-8138.14734
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Shimizu Hiroshi
中科院分区：
文献类型：
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作者：
Sugai Tatsuro;Ujiie Hideyuki;Nakamura Hiroyuki;Kikuchi Kazuhiro;Iwata Hiroaki;Shimizu Hiroshi
图1所示。临床、组织学和免疫学结果。(a)下肢水肿性红斑病变和糜烂。黄色箭头表示紧张的水泡。(b)蓝色箭头显示左腿结痂和糜烂。(c)臀部环状红斑病变和糜烂。(d)左腿水肿性红斑的小泡组织病理学检查显示表皮下（黄色箭头）和表皮内（蓝色箭头）的混合特征（苏木精-伊红[HE]，原始放大倍数980）。(e)在水疱中观察到嗜中性粒细胞和嗜酸性粒细胞浸润和棘囊溶解细胞（黑色箭头）（HE, 9400）。(f)直接免疫荧光（DIF）显示免疫球蛋白（Ig） G在基膜区（BMZ）（白色箭头）和表皮细胞间隙（ICS）的沉积。(g) DIF显示C3在BMZ（白色箭头）和ICS的沉积。(h)使用1 mol/L nacl分裂正常人皮肤的间接IF显示患者IgG对表皮侧（白色箭头）和ICS（黄色箭头）的反应性。(i)使用人表皮提取物的免疫印迹显示，IgG对160 kda蛋白（对应于粘粒蛋白1）和230 kda蛋白（对应于BP230）起反应。BP，大疱性类天疱疮；寻常性天疱疮。
Figure 1. Clinical, histological and immunological findings.(a) Edematous erythematous lesions and erosions on the lower limbs. Yellow arrowheads indicate a tense blister.(b) Blue arrowheads show crusts and erosions on the left leg.(c) Annular erythematous lesions and erosions on the buttocks.(d) Histopathological examination taken from a small vesicle on edematous erythema on the left leg reveal the mixed features of subepidermal blisters (yellow arrows) and intraepidermal blisters (blue arrows)(hematoxylin–eosin [HE], original magnification 980).(e) Neutrophilic and eosinophilic infiltration and acantholytic cells (black arrows) are observed in a blister (HE, 9400).(f) Direct immunofluorescence (DIF) shows the deposition of immunoglobulin (Ig) G at the basement membrane zone (BMZ)(white arrowheads) and in the intercellular space of the epidermis (ICS).(g) DIF shows the deposition of C3 at the BMZ (white arrowheads) and in the ICS.(h) Indirect IF using 1 mol/L NaCl-split normal human skin shows reactivity of the patient’s IgG to the epidermal side (white arrowheads) and the ICS (yellow arrows).(i) Immunoblotting using human epidermal extracts shows IgG reacting to a 160-kDa protein (corresponding to desmoglein 1) and to a 230-kDa protein (corresponding to BP230). BP, bullous pemphigoid; PV, pemphigus vulgaris.