Schulze F, Kasperkiewicz M, Zillikens D, Schmidt E
Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland,
Hautarzt. 2013 Dec;64(12):931-43; quiz 944-5. doi: 10.1007/s00105-013-2599-9.
Pemphigoid diseases are a group of autoimmune disorders characterized by subepidermal blistering and autoantibodies against structural proteins of the dermal-epidermal junction. In bullous pemphigoid, the most common subepidermal blistering autoimmune disease, antibodies are directed against the hemidesmosomal antigens BP180 (collagen type XVII) and BP230. Bullous pemphigoid typically presents with severe pruritus and tense blisters accompanied by erosions and crusts in elderly patients. Diagnostic landmarks are the detection of linear IgG and/or C3 deposits at the dermo-epidermal junction by direct immunofluorescence microscopy of a perilesional biopsy and the detection of serum autoantibodies by indirect immunofluorescence microscopy on human salt-split skin and ELISA employing recombinant immunodominant fragments of BP180 and BP230. Treatment options include topical (class IV) and/or systemic corticosteroids, frequently combined with immunomodulatory agents like dapsone and tetracyclines or immunosuppressants such as methotrexate and azathioprine.
类天疱疮疾病是一组自身免疫性疾病，其特征为表皮下水疱形成以及针对真皮-表皮交界处结构蛋白的自身抗体。在最常见的表皮下水疱形成的自身免疫性疾病大疱性类天疱疮中，抗体针对半桥粒抗原BP180（XVII型胶原蛋白）和BP230。大疱性类天疱疮通常在老年患者中表现为严重瘙痒和紧张性水疱，并伴有糜烂和结痂。诊断标志是通过对皮损周围活检组织进行直接免疫荧光显微镜检查，在真皮-表皮交界处检测到线性IgG和/或C3沉积，以及通过对人盐裂皮肤进行间接免疫荧光显微镜检查和使用BP180和BP230重组免疫显性片段的ELISA检测血清自身抗体。治疗选择包括局部（IV级）和/或全身皮质类固醇，通常与免疫调节剂如氨苯砜和四环素或免疫抑制剂如甲氨蝶呤和硫唑嘌呤联合使用。
