Parallel fluctuation of anti-desmoglein 3 and anti-BP180 autoantibody titres in a patient with bullous pemphigoid

Parallel fluctuation of anti-desmoglein 3 and anti-BP180 autoantibody titres in a patient with bullous pemphigoid
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DOI:
10.1111/j.1365-2230.2004.01598.x
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发表时间:
2004-11-01
影响因子:
4.1
通讯作者:
Nishikawa, T
Nishikawa, T
中科院分区:
医学4区
文献类型:
--
作者:
Takahashi, H;Anzai, H;Nishikawa, T

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我们报告了一位73岁的日本女性,她产生了抗BP180和桥粒芯糖蛋白3(Dsg3)的IgG自身抗体。她表现出四肢上的紧张性水疱,没有明显的粘膜受累,皮肤活检表明表皮下水疱伴嗜酸性海绵状增生。她的临床和组织学特征表明诊断为大疱性类天疱疮,而抗Dsg3 IgG可能没有显示出明显的致病作用。有趣的是,在整个过程中,抗Dsg3 IgG的滴度与抗BP180 IgG的滴度平行波动,具有两个耀斑。虽然自身抗体产生的确切机制仍不清楚,但针对两种独立皮肤抗原的IgG自身抗体产生的密切联系表明针对皮肤自身抗原的共同免疫调节机制。
We report a 73-year-old Japanese female who developed IgG autoantibodies against BP180 as well as desmoglein 3 (Dsg3). She showed tense blisters on the extremities without apparent mucosal involvement and a skin biopsy indicated subepidermal blisters with eosinophilic spongiosis. Her clinical and histologic features indicated the diagnosis of bullous pemphigoid while anti-Dsg3 IgG might not show an apparent pathogenic effect. Interestingly, titres of anti-Dsg3 IgG fluctuated in parallel with those of anti-BP180 IgG throughout the course with two flares. Although the exact mechanism for autoantibody production is still unknown, the close link in the production of IgG autoantibodies against two independent skin antigens suggests a shared immunoregulatory mechanism against cutaneous autoantigens.