Caproni M, Rolfo S, Bernacchi E, Bianchi B, Brazzini B, Fabbri P
II degrees Clinica Dermatologica, Università degli Studi di Firenze, Via della Pergola 58, 50121 Florence, Italy.
Br J Dermatol. 1999 Jun;140(6):1072-8. doi: 10.1046/j.1365-2133.1999.02904.x.
Linear IgA bullous dermatosis (LAD) is an acquired, heterogeneous, subepidermal blistering disease characterized by linear IgA deposits at the dermoepidermal basement membrane zone (BMZ), often with circulating IgA antibodies to the BMZ. The pathogenetic mechanism, possibly related to the immunophenotype of infiltrating cells, as well as the potential role of cytokines in determining bullous lesions, have not yet been elucidated. An immunohistochemical study was performed with a large panel of monoclonal antibodies [to CD3, CD4, CD8, CD25, CD1a, CD30, CD54, CD50, endothelial leucocyte adhesion molecule-1, vascular cell adhesion molecule-1, myeloperoxidase (MPO), eosinophil cationic protein EG1 and EG2, tryptase, HLA-DR, human interleukin (IL)-3, human IL-5, human IL-8, human IL-4, tumour necrosis factor (TNF)-alpha, interferon (IFN)-gamma and granulocyte/macrophage colony-stimulating factor] using the alkaline phosphatase-antialkaline phosphatase procedure on lesional and perilesional skin of nine patients (one male, eight female; age range 8 months-80 years) with clinical, histological and immunofluorescent proven LAD. The predominant infiltrating cells, distributed mostly inside and below the bullae, were neutrophils and eosinophils which showed intense activation (MPO +, EG1 +, EG2 +). The lymphocytic infiltrate, consisting principally of CD4 +, HLA-DR + and CD30 + T cells, had a predominantly perivascular distribution. Proinflammatory cytokines, such as TNF-alpha and IFN-gamma, showed a moderate focal expression on the dermal perivascular sites; IL-8 was found to have a particularly intense staining on all the epidermal cell layers and at perivascular and vascular sites. Other cytokines, such as IL-4 and IL-5, showed a prevalent intracytoplasmic staining on some cells of the dermal infiltrate (probably mastocytes and lymphocytes), and at the dermal-epidermal separation sites there was also an intense scattered distribution of IL-5. The specific tissue lesions of LAD may be the consequence of the IgA deposits at the BMZ and also of the release of these cytokines together with tissue damage enzymes derived from neutrophils or eosinophils.
线状IgA大疱性皮肤病（LAD）是一种获得性、异质性、表皮下大疱性疾病，其特征为在真皮表皮基底膜带（BMZ）出现线状IgA沉积，常伴有针对BMZ的循环IgA抗体。其发病机制，可能与浸润细胞的免疫表型有关，以及细胞因子在决定大疱性损害中的潜在作用，尚未阐明。我们使用碱性磷酸酶 - 抗碱性磷酸酶方法，对9例（1例男性，8例女性；年龄范围8个月至80岁）经临床、组织学和免疫荧光证实为LAD的患者的皮损及皮损周围皮肤，用一大组单克隆抗体[针对CD3、CD4、CD8、CD25、CD1a、CD30、CD54、CD50、内皮白细胞黏附分子 - 1、血管细胞黏附分子 - 1、髓过氧化物酶（MPO）、嗜酸性粒细胞阳离子蛋白EG1和EG2、类胰蛋白酶、HLA - DR、人白细胞介素（IL） - 3、人IL - 5、人IL - 8、人IL - 4、肿瘤坏死因子（TNF） - α、干扰素（IFN） - γ和粒细胞/巨噬细胞集落刺激因子]进行了免疫组织化学研究。主要的浸润细胞大多分布在水疱内及水疱下方，为中性粒细胞和嗜酸性粒细胞，它们显示出强烈的活化（MPO +、EG1 +、EG2 +）。淋巴细胞浸润主要由CD4 +、HLA - DR +和CD30 + T细胞组成，主要呈血管周围分布。促炎细胞因子，如TNF - α和IFN - γ，在真皮血管周围部位呈中度局灶性表达；发现IL - 8在所有表皮细胞层以及血管周围和血管部位有特别强烈的染色。其他细胞因子，如IL - 4和IL - 5，在真皮浸润的一些细胞（可能是肥大细胞和淋巴细胞）上呈普遍的胞质内染色，并且在真皮 - 表皮分离部位也有IL - 5的强烈散在分布。LAD的特异性组织损害可能是BMZ处IgA沉积的结果以及这些细胞因子与源自中性粒细胞或嗜酸性粒细胞的组织损伤酶共同释放的结果。
