Kersting E, Goebeler M, Hamm H, Rose C, Zillikens D, Bröcker E B
Klinik und Poliklinik für Haut- und Geschlechtskrankheiten, Universität Würzburg.
Hautarzt. 2000 May;51(5):340-4. doi: 10.1007/s001050051129.
A 29-year-old female patient with an autoimmune subepidermal blistering disease had linear deposits of both IgA and IgG at the basement membrane zone. Clinically, the patient presented with tense blisters on the face, trunk, extremities and oral mucosa. Histologically, we found a subepidermal blister formation and a predominantly neutrophilic infiltrate. Direct immunofluorescence showed linear deposits of IgA along the basement membrane zone, as well as linear deposits of IgG and C3 as typically found in bullous pemphigoid. Indirect immunofluorescence demonstrated circulating IgA and IgG autoantibodies. This case extends previous reports on a subgroup of patients with subepidermal blistering diseases characterized by the presence of both IgA and IgG anti-basement membrane antibodies. These patients reveal clinical, histological and immunopathological features of linear IgA disease and bullous pemphigoid.
一名患有自身免疫性表皮下大疱病的29岁女性患者，在基底膜带出现IgA和IgG的线性沉积。临床上，患者面部、躯干、四肢及口腔黏膜出现紧张性水疱。组织学上，我们发现表皮下疱形成，且主要为嗜中性粒细胞浸润。直接免疫荧光显示沿基底膜带有IgA的线性沉积，以及如大疱性类天疱疮中典型所见的IgG和C3的线性沉积。间接免疫荧光显示循环IgA和IgG自身抗体。该病例扩展了先前关于以同时存在IgA和IgG抗基底膜抗体为特征的表皮下大疱病亚组患者的报道。这些患者呈现出线性IgA疾病和大疱性类天疱疮的临床、组织学和免疫病理学特征。
