Bullous pemphigoid - 大皰性類天皰瘡
大皰性類天皰瘡 (Bullous pemphigoid) 是指會產生大皰的各種皮膚疾病。「大皰性類天皰瘡」是一種自體免疫性、伴有搔癢的皮膚病，常見於60歲以上的長者，會形成水皰。
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。
relevance score : -100.0%
ReferencesPemphigus 和 bullous pemphigoid 是由自體抗體引起水皰的皮膚疾病。 在 pemphigus 中，表皮層及黏膜的角化細胞失去黏附能力；而在 bullous pemphigoid 中，皮膚基底層的細胞與下層結構的連結受損。 Pemphigus 的水皰是由自身抗體直接作用所致，bullous pemphigoid 則是自體抗體透過活化補體引發發炎。已鑑定的自體抗體靶向蛋白質包括：pemphigus 中的橋粒核心糖蛋白（參與細胞黏附）以及 bullous pemphigoid 中的半橋粒蛋白（將細胞錨定於基底膜下層）。
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，主要影響老年人。近幾十年病例增加，與人口老化、藥物相關事件以及非大皰性疾病診斷方法的改進有關。疾病涉及 T 細胞反應異常，產生針對特定蛋白質（BP180 和 BP230）的自身抗體（IgG、IgE），導致發炎與皮膚支撐結構受損。常見症狀為身體及四肢出現凸起、發癢的斑塊並形成水泡，黏膜受累較少。治療以強效局部或全身類固醇為主，近年研究亦強調 doxycycline、dapsone 及 immunosuppressants 的療效與安全性，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.
