Overlap of IgA pemphigus and linear IgA dermatosis in a patient with ulcerative colitis: a mere coincidence?
Overlap of IgA pemphigus and linear IgA dermatosis in a patient with ulcerative colitis: a mere coincidence?
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溃疡性结肠炎患者的 IgA 天疱疮和线性 IgA 皮肤病重叠：仅仅是巧合吗？
DOI：
10.2340/00015555-1658
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发表时间：
2014
影响因子：
3.6
通讯作者：
S. Hofmann
中科院分区：
文献类型：
--
作者：
J. Kern;W. Gehring;W. Kreisel;M. Hertl;K. Technau;L. Bruckner;S. Hofmann
伊加天疱疮和线性伊加皮肤病（LAD）是罕见的自身免疫性大疱性疾病，其特征分别是表皮内或真皮-表皮伊加沉积和水疱形成。伊加天疱疮根据临床和组织学表现分为不同的亚组：角膜下脓疱性皮肤病（SPD）型和表皮内嗜酸性（IEN）型（1-3）。后者的临床特征是扁平水疱性脓疱形成环形病变，中央有痂，常表现为“向日葵样”病变。直接免疫荧光显示整个表皮细胞间伊加沉积。SPD型患者经常显示针对桥粒蛋白桥粒胶蛋白1的伊加类的循环自身抗体，这与针对包括桥粒胶蛋白1或3的异质性抗原的伊加自身抗体在具有IEN型伊加天疱疮的患者中形成对比。表皮下大疱性皮肤病LAD表现为环形紧张性水疱，具有“宝石冠状”模式，基底膜区存在线性伊加沉积，循环自身抗体最常靶向可溶性BP 180/胶原XVII胞外域（4）。与更常见的富含嗜酸性粒细胞的自身免疫性水疱性皮肤病寻常性天疱疮和大疱性类天疱疮相比，伊加天疱疮和LAD的发生基于皮肤中的伊加沉积，这导致中性粒细胞趋化性和随后的水疱形成（5）。病例报告
IgA pemphigus and linear IgA dermatosis (LAD) are rare autoimmune bullous disorders characterized by intraepidermal or dermo-epidermal IgA deposition and blister formation, respectively. IgA pemphigus is divi-ded into different subgroups according to clinical and histological findings: a subcorneal pustular dermatosis (SPD) type and an intraepidermal neutrophilic (IEN) type (1–3). The latter is clinically characterized by flac-cid vesiculopustules forming annular lesions with central crusts, often appearing as “sunflower-like” lesions. Direct immunofluorescence demonstrates intercellular IgA de-position throughout the epidermis. Patients with the SPD type often display circulating autoantibodies of the IgA class against the desmosomal protein desmocollin 1, in contrast to IgA autoantibodies to heterogeneous antigens including desmoglein 1 or 3 in patients with the IEN type of IgA pemphigus. The subepidermal bullous dermatosis LAD presents with annular tense blisters with a “crown of jewels-like” pattern, linear IgA deposition at the ba-sement membrane zone, and circulating autoantibodies that most frequently target the soluble BP180/collagen XVII ectodomain (4). In contrast to the more common eosinophil-rich autoimmune blistering dermatoses pem -phigus vulgaris and bullous pemphigoid, the development of IgA pemphigus and LAD is based on IgA deposition in the skin, which results in neutrophil chemotaxis and subsequent blister formation (5). CASE REPORT