Concurrence of bullous pemphigoid and herpetiform pemphigus with igg antibodies to desmogleins 1/3 and desmocollins 1-3.
Concurrence of bullous pemphigoid and herpetiform pemphigus with igg antibodies to desmogleins 1/3 and desmocollins 1-3.
复制标题
大疱性类天疱疮和疱疹样天疱疮同时伴有桥粒芯蛋白 1/3 和桥粒芯蛋白 1-3 的 igg 抗体。
DOI：
10.1111/bjd.12019
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发表时间：
2012
期刊：
影响因子：
--
通讯作者：
Hashimoto T.
中科院分区：
文献类型：
--
作者：
Ohata C;Koga H;Teye K;Ishii N;Hamada T;Dainichi T;Furumura M;Sato M;Sueki H;Hashimoto T.
女士，天疱疮中的自身抗体优先针对桥粒芯糖蛋白1(Dsg1)和Dsg3，很少针对桥粒芯糖蛋白1-3(Dsc1-3)。1疱疹样天疱疮是天疱疮的一个亚型，以周围有水泡的瘙痒状环形红斑、少见的粘膜受累和组织病理学确定的嗜酸性海绵样变为特征。最近发现1例寻常型天疱疮(PV)皮损是由于免疫球蛋白G(Ig G)抗Dsc3自身抗体引起的。2在这项研究中，我们报告了第一例同时患有大疱性类天疱疮(BP)和PH并同时具有DGS和DSC抗体的病例。患者为83岁女性，有3个月的口腔酸蚀和溃疡病史。这位病人的病史并不引人注目。体格检查发现舌头、软腭和颊粘膜有多个水泡、侵蚀和溃疡(图1a)。10天后，瘙痒性荨麻疹环状红斑出现在腹部和下肢，其中一些在周围出现小水泡(图1B)。实验室检查显示轻度嗜酸性粒细胞增多症。右下肢活检标本显示嗜酸性海绵体和许多表皮内小泡，但表皮内无明显棘层松解(图1C)，也可见少数表皮下小泡，有许多嗜酸性浸润物(图1D)。直接免疫荧光显示整个表皮角质形成细胞表面有IgG沉积(图2a)，C3沉积在表皮基底膜区。
MADAM, Autoantibodies in pemphigus preferentially target desmoglein 1 (Dsg1) and Dsg3, and rarely desmocollins 1–3 (Dsc1–3). 1 Pemphigus herpetiformis (PH) is a subtype of pemphigus and is characterized by pruritic annular erythemas with vesicles in the periphery, rarity of mucosal involvement and histopathologically determined eosinophilic spongiosis. Recently, it was suggested that immunoglobulin G (IgG) anti-Dsc3 autoantibodies caused skin lesion in a case of pemphigus vulgaris (PV). 2 In this study, we report the first case of concurrent bullous pemphigoid (BP) and PH with IgG antibodies to both Dsgs and Dscs.An 83-year-old woman presented with a 3-month history of painful oral erosions and ulcers. The patient’s medical history was unremarkable. Physical examination revealed multiple blisters, erosions and ulcers on the tongue, soft palate and buccal mucosa (Fig. 1a). Ten days later, pruritic urticarial annular erythemas developed on the abdomen and lower extremities, some of which showed small vesicles at the periphery (Fig. 1b). Laboratory examination revealed slight hypereosinophilia. A biopsy specimen from the right lower extremity revealed eosinophilic spongiosis and many intraepidermal vesicles without apparent acantholysis in the epidermis (Fig. 1c), and also a few subepidermal vesicles with many eosinophilic infiltrates (Fig. 1d). Direct immunofluorescence showed IgG deposition on the cell surfaces of keratinocytes in the whole epidermis (Fig. 2a), and C3 deposition to the epidermal basement membrane zone