Teraki Y, Amagai N, Hashimoto T, Kusunoki T, Nishikawa T
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Arch Dermatol. 1991 Feb;127(2):221-4. doi: 10.1001/archderm.127.2.221.
We describe a 7-year-old girl with recurrent pruritic vesiculopustular lesions involving the trunk, extremities, face, and oral mucosa. Histopathologic examination revealed intraepidermal bullae containing neutrophils and eosinophils, and direct immunofluorescence test showed the deposition of IgA in the intercellular space of the epidermis. Circulating IgA anti-intercellular antibodies were also detected by indirect immunofluorescence test. Immunofluorescence studies using monoclonal antibodies to human IgA subclasses showed that these IgA antibodies belonged to IgA1. Antisera against J chain and secretory component did not show any specific intercellular staining. Surface IgA(+)-B cells were transiently increased in the peripheral blood during the active stage of the disease. These results indicated the extragut origin of these IgA antibodies. Dapsone therapy was shown to be very effective.
我们描述了一名7岁女童，其躯干、四肢、面部及口腔黏膜反复出现瘙痒性水疱脓疱性皮损。组织病理学检查显示表皮内水疱，疱内含中性粒细胞和嗜酸性粒细胞，直接免疫荧光试验显示表皮细胞间有IgA沉积。间接免疫荧光试验也检测到循环中的IgA抗细胞间抗体。使用针对人IgA亚类的单克隆抗体进行的免疫荧光研究表明，这些IgA抗体属于IgA1。抗J链和分泌成分的抗血清未显示任何特异性细胞间染色。在疾病活动期，外周血中表面IgA(+) - B细胞短暂增加。这些结果表明这些IgA抗体起源于肠道外。氨苯砜治疗显示非常有效。
