Wojnarowska F, Bhogal B S, Black M M
Department of Dermatology, Churchill Hospital, Oxford, U.K.
Br J Dermatol. 1994 Aug;131(2):201-4. doi: 10.1111/j.1365-2133.1994.tb08491.x.
Linear IgA disease is characterized by the presence of linear IgA deposits at the basement membrane zone of the skin, and in some cases by circulating basement membrane zone antibodies. The disease occurs in both adults and children, and is designated adult linear IgA disease in the former and chronic bullous disease of childhood in the latter. The subclass distribution of the circulating and bound basement membrane zone antibodies was studied in 32 children and eight adults. The results were compared with five dermatitis herpetiformis patients and five normal controls. The circulating antibodies (39 patients) and the cutaneous deposits (39 patients) were IgA1 in all 40 patients with linear IgA disease. The cutaneous deposits in dermatitis herpetiformis were also all IgA1, and no circulating antibodies were detected. The controls were all negative. This large series of children and adults with linear IgA disease demonstrates that the circulating and cutaneous basement membrane zone deposits are all IgA1, and suggests that linear IgA disease is an IgA1-mediated disease.
线状IgA疾病的特征是在皮肤基底膜区出现线状IgA沉积，在某些情况下还存在循环性基底膜区抗体。该疾病在成人和儿童中均有发生，前者被称为成人线状IgA疾病，后者则称为儿童慢性大疱性疾病。对32名儿童和8名成人的循环性和结合性基底膜区抗体的亚类分布进行了研究。将结果与5名疱疹样皮炎患者和5名正常对照进行了比较。在所有40例线状IgA疾病患者中，循环抗体（39例患者）和皮肤沉积物（39例患者）均为IgA1。疱疹样皮炎的皮肤沉积物也均为IgA1，且未检测到循环抗体。对照组均为阴性。这一大系列的儿童和成人线状IgA疾病表明，循环性和皮肤基底膜区沉积物均为IgA1，并提示线状IgA疾病是一种由IgA1介导的疾病。
