Beutner E H, Chorzelski T P, Wilson R M, Kumar V, Michel B, Helm F, Jablonska S
Department of Microbiology, University of Buffalo, SUNY 14214.
J Am Acad Dermatol. 1989 Jan;20(1):89-97. doi: 10.1016/s0190-9622(89)70014-1.
The cases of two patients with vesiculobullous lesions were diagnosed clinically and histopathologically as pemphigus foliaceus; unexpectedly, both revealed intercellular IgA, but not IgG, in the upper epidermis by direct immunofluorescence. Such histologic and immunofluorescence findings have been reported in eight other cases. In our cases no circulating IgA or IgG intercellular antibodies could be detected; in four of eight other reported cases IgA antibodies showed intercellular staining like that of pemphigus antibodies. Subcorneal acantholytic lesions occurred in both our cases; of the other cases reported, five had essentially identical histopathologic findings. The clinical and histopathologic features of pemphigus, as well as the recent findings of circulating IgA intercellular antibodies alone or with IgG antibodies, appear to place this disease into the spectrum of pemphigus. The 10 IgA pemphigus cases reported to date fall into one of two groups, the IgA pemphigus foliaceus (including our two cases) and IgA pemphigus of the intraepidermal neutrophilic type, which seems to be less common.
两名患有水疱大疱性皮损的患者经临床和组织病理学诊断为落叶型天疱疮；出乎意料的是，通过直接免疫荧光法，二者的表皮上层均显示细胞间IgA，而非IgG。另外8例也报告了此类组织学和免疫荧光结果。在我们的病例中，未检测到循环IgA或IgG细胞间抗体；在其他报告的8例病例中，有4例的IgA抗体显示出与天疱疮抗体类似的细胞间染色。我们的两个病例均出现了角层下棘层松解性损害；在其他报告的病例中，有5例具有基本相同的组织病理学表现。天疱疮的临床和组织病理学特征，以及最近单独出现循环IgA细胞间抗体或与IgG抗体同时出现的发现，似乎将这种疾病纳入了天疱疮的范畴。迄今为止报告的10例IgA天疱疮病例可分为两组，即落叶型IgA天疱疮（包括我们的两例）和表皮内嗜中性粒细胞型IgA天疱疮，后者似乎较为少见。
