Bullous pemphigoid - 大疱性类天疱疮https://en.wikipedia.org/wiki/Bullous_pemphigoid
大疱性类天疱疮 (Bullous pemphigoid) 指诱发大疱的各种皮肤病。 “大疱性类天疱疮”是一种自身免疫性瘙痒性皮肤病,好发于 60 岁以上的老年人。在大疱性类天疱疮中观察到表皮和真皮层之间的空间形成水疱。

☆ 德国 Stiftung Warentest 2022 年的结果显示,消费者对 ModelDerm 的满意度仅略低于付费远程医疗咨询。
  • 一张照片显示腿上长满了破裂的水泡,这会影响整个身体。
  • Pemphgoid vulgaris 在老年人中更常见。
  • 最初的症状有时是荨麻疹。
References Mechanisms of Disease: Pemphigus and Bullous Pemphigoid 26907530 
NIH
Pemphigus 和 bullous pemphigoid 是由于自身抗体而形成水疱的皮肤病。在 pemphigus 中,外层皮肤层和粘膜中的细胞失去了粘在一起的能力,而在 pemphigoid 中,皮肤底部的细胞失去了与下层的连接。 pemphigus 的水疱是由自身抗体直接引起的,而 pemphigoid 是自身抗体通过激活补体引发炎症。这些自身抗体靶向的特定蛋白质已被鉴定: pemphigus 中的桥粒芯糖蛋白(参与细胞粘附)和 pemphigoid 中的半桥粒中的蛋白质(将细胞锚定到下层)。
Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane).
 Bullous pemphigoid 31090818 
NIH
Bullous pemphigoid 是最常见的自身免疫性大疱性疾病,通常影响老年人。近几十年来病例的增加与人口老龄化、毒品相关事件以及非大疱性病症诊断方法的改进有关。它涉及 T 细胞反应故障和针对特定蛋白质(BP180 和 BP230)的自身抗体(IgG 和 IgE)的产生,导致炎症和皮肤支持结构的破坏。症状通常包括身体和四肢上凸起、发痒的斑块起水泡,很少累及粘膜。治疗主要依靠强效的局部和全身类固醇,最近的研究强调了其他疗法的益处和安全性,例如 doxycycline, dapsone, and immunosuppressants ,旨在减少类固醇的使用。
Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.