Kawahara Y, Hashimoto T, Watanabe K, Kurihara S, Matsuo I, Nishikawa T
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
J Dermatol. 1996 Apr;23(4):254-8. doi: 10.1111/j.1346-8138.1996.tb04008.x.
Patients showing coexistent linear IgG and IgA deposition along the basement membrane zone on direct immunofluorescence have been described as either bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA bullous dermatosis, or cicatricial pemphigoid, depending on the clinical features and laboratory findings. In the present report, we describe two cases showing atypical clinical features distinct from those of other known bullous diseases. No circulating antibodies were detected by indirect immunofluorescence of normal human skin. Indirect immunofluorescence of 1 M NaCl split skin revealed IgG and/or IgA antibodies reactive with the dermal side of the split. Immunoblotting of normal human epidermal and dermal extracts showed no apparent reactivity with known autoantigens. The results suggest that there may be a unique and distinct bullous disease with linear IgG and IgA deposition at the basement membrane zone.
根据临床特征和实验室检查结果，在直接免疫荧光检查中显示沿基底膜带同时存在线性IgG和IgA沉积的患者，被诊断为大疱性类天疱疮、获得性大疱性表皮松解症、线性IgA大疱性皮肤病或瘢痕性类天疱疮。在本报告中，我们描述了两例具有不同于其他已知大疱性疾病的非典型临床特征的病例。正常人皮肤间接免疫荧光检查未检测到循环抗体。1M NaCl分离皮肤的间接免疫荧光显示IgG和/或IgA抗体与分离皮肤的真皮侧反应。正常人表皮和真皮提取物的免疫印迹显示与已知自身抗原无明显反应。结果表明，可能存在一种在基底膜带具有线性IgG和IgA沉积的独特的大疱性疾病。
