Hintner H, Schuler G, Fritsch P
Hautarzt. 1982 Jun;33(6):310-4.
In a patient with epidermolysis bullosa acquisita the characteristic dermolytic cleavage was demonstrated by electron microscopy and by mapping of antigenic determinants (type IV collagen, laminin, bullous pemphigoid antigen) of the dermal-epidermal junction. The latter method represents a rapid and reliable way to determine the cleavage plane in diseases which display subepidermal blister formation at the light-microscopic level. The classification of epidermolysis bullosa acquisita is still under dispute. Due to its highly characteristic clinical, ultrastructural, and immunologic features and pending further experimental data, epidermolysis bullosa acquisita should be regarded as a separate disease entity; its lumping together with cicatricial pemphigoid, as proposed by some authors appears speculative. Therapy of epidermolysis bullosa acquisita is generally regarded as difficult; following a 3-year course of high dose vitamin E therapy our patient underwent complete clearing; the possibility of a spontaneous remission, on the other hand, cannot be unequivocally ruled out.
在一位获得性大疱性表皮松解症患者中，通过电子显微镜以及对真皮 - 表皮连接处抗原决定簇（IV型胶原、层粘连蛋白、大疱性类天疱疮抗原）的定位，证实了特征性的真皮溶解性分离。后一种方法是在光镜水平显示表皮下疱形成的疾病中确定分离平面的快速且可靠的方法。获得性大疱性表皮松解症的分类仍存在争议。由于其具有高度特征性的临床、超微结构和免疫学特征，且有待进一步的实验数据，获得性大疱性表皮松解症应被视为一种独立的疾病实体；一些作者提议将其与瘢痕性类天疱疮归为一类，这似乎是推测性的。获得性大疱性表皮松解症的治疗通常被认为是困难的；在接受了3年高剂量维生素E治疗后，我们的患者完全治愈；另一方面，不能明确排除自发缓解的可能性。
