Bullous pemphigoid - 大皰性類天皰瘡
大皰性類天皰瘡 (Bullous pemphigoid) 指誘發大皰的各種皮膚病。 「大皰性類天皰瘡」是一種自體免疫性搔癢性皮膚病，好發於60 歲以上的老年人。水皰。
一張照片顯示腿上長滿了破裂的水泡，這會影響整個身體。
最初的症狀有時是蕁麻疹。
References
Pemphigus 和 bullous pemphigoid 是由於自體抗體而形成水皰的皮膚病。在 pemphigus 中，外層皮膚層和黏膜中的細胞失去了黏在一起的能力，而在 pemphigoid 中，皮膚底部的細胞失去了與下層的連結。 pemphigus 的水皰是由自身抗體直接引起的，而 pemphigoid 是自體抗體透過活化補體引發發炎。這些自體抗體靶向的特定蛋白質已被鑑定： pemphigus 中的橋粒芯糖蛋白（參與細胞黏附）和 pemphigoid 中的半橋粒中的蛋白質（將細胞錨定到下層）。
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).
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 是最常見的自體免疫性大皰性疾病，通常影響老年人。近幾十年來病例的增加與人口老化、毒品相關事件以及非大皰性病症診斷方法的改進有關。它涉及 T 細胞反應故障和針對特定蛋白質（BP180 和 BP230）的自身抗體（IgG 和 IgE）的產生，導致發炎和皮膚支持結構的破壞。症狀通常包括身體和四肢上凸起、發癢的斑塊起水泡，很少涉及黏膜。治療主要依靠強效的局部和全身類固醇，最近的研究強調了其他療法的益處和安全性，例如 doxycycline, dapsone, and immunosuppressants ，旨在減少類固醇的使用。
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.
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.
