Zillikens D
Department of Dermatology, University of Würzburg, Germany.
J Dermatol Sci. 1999 Jun;20(2):134-54. doi: 10.1016/s0923-1811(99)00019-5.
The hemidesmosome is a membrane-associated supramolecular dermal epidermal complex linking the cytoskeleton of the basal keratinocyte to structures within the papillary dermis. Different components of this complex have been identified as autoantigens in autoimmune bullous skin diseases. Some of the autoantigens have been characterized at the molecular level. Little is known, however, about the factors that initiate the production of autoantibodies. By histopathology, acquired skin diseases of hemidesmosomes show subepidermal blisters and by direct immunofluorescence, linear deposits of IgG, C3 or IgA at the dermal epidermal junction. Bullous pemphigoid (BP) is the most common acquired disease of hemidesmosomes. Two proteins, BP180 and BP230, have been identified as primary targets of autoantibodies in BP. In addition, pemphigoid/herpes gestationis, lichen planus pemphigoides, cicatricial pemphigoid and linear IgA disease are characterized by an immune response to BP180. Laminin 5 is another well-characterized anchoring filament-lamina densa component of hemidesmosomes. Patients with autoantibodies to laminin 5 show the clinical phenotype of cicatricial pemphigoid. Other acquired skin diseases of the hemidesmosomes reveal autoantibodies to a plectin-like protein, the beta4 subunit of alpha6beta4 integrin, uncein and a not yet characterized 168 kDa protein. Recently, diseases with autoantibodies to 105 and 200 kDa proteins of the lower lamina lucida have been reported. The association of these autoantigens with hemidesmosomes still needs to be demonstrated. Finally, anchoring fibrils associate with the dermal epidermal anchoring complex. The major structural component of anchoring fibrils is type VII collagen, the autoantigen of epidermolysis bullosa acquisita.
半桥粒是一种与膜相关的超分子真皮表皮复合体，它将基底角质形成细胞的细胞骨架与乳头层真皮内的结构相连。该复合体的不同成分已被确定为自身免疫性大疱性皮肤病中的自身抗原。其中一些自身抗原已在分子水平上得到表征。然而，关于引发自身抗体产生的因素却知之甚少。通过组织病理学检查，获得性半桥粒皮肤病表现为表皮下水疱，通过直接免疫荧光检查，在真皮表皮交界处可见IgG、C3或IgA的线性沉积。大疱性类天疱疮（BP）是最常见的获得性半桥粒皮肤病。两种蛋白质，BP180和BP230，已被确定为BP中自身抗体的主要靶标。此外，妊娠类天疱疮/疱疹样皮炎、扁平苔藓样类天疱疮、瘢痕性类天疱疮和线状IgA大疱性皮病的特征是对BP180产生免疫反应。层粘连蛋白5是半桥粒另一种特征明确的锚定细丝-致密板成分。针对层粘连蛋白5产生自身抗体的患者表现出瘢痕性类天疱疮的临床表型。其他获得性半桥粒皮肤病显示针对一种类似网蛋白的蛋白质、α6β4整合素的β4亚基、uncein和一种尚未表征的168 kDa蛋白质的自身抗体。最近，已报道了针对透明层下部105和200 kDa蛋白质产生自身抗体的疾病。这些自身抗原与半桥粒的关联仍需证实。最后，锚定原纤维与真皮表皮锚定复合体相关。锚定原纤维的主要结构成分是VII型胶原，即获得性大疱性表皮松解症的自身抗原。
