Bullous pemphigoid - 大疱性类天疱疮
大疱性类天疱疮 (Bullous pemphigoid) 指一类可导致大疱形成的皮肤疾病。 “大疱性类天疱疮”是一种自身免疫性、伴瘙痒的皮肤病，好发于 60 岁以上的老年人。在该病中，可见表皮与真皮层之间形成水疱。
一张照片显示，腿上长满了破裂的水泡，这可能影响整个身体。
最初的症状有时表现为荨麻疹。
relevance score : -100.0%
ReferencesPemphigus 和 bullous pemphigoid 是由自身抗体导致水疱形成的皮肤疾病。 在 Pemphigus 中，表皮层及黏膜的细胞失去相互粘附的能力；而在 bullous pemphigoid 中，皮肤底层的细胞失去与下层的连接。 Pemphigus 的水疱直接由自身抗体介导，bullous pemphigoid 的水疱则是自身抗体通过激活补体引发炎症所致。这些自身抗体靶向的特定蛋白已被鉴定：Pemphigus 中的桥粒芯糖蛋白（参与细胞粘附）和 bullous 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).
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 是最常见的自身免疫性大疱性疾病，主要发生在老年人。近几十年来，病例增多与人口老龄化、药物相关因素以及非大疱性疾病诊断技术的进步有关。该病涉及 T 细胞功能失调，并产生针对特定蛋白质（BP180 和 BP230）的自身抗体（IgG、IgE），导致炎症和皮肤附属结构受损。临床表现常为躯干和四肢出现隆起、伴瘙痒的斑块并形成水泡，粘膜受累较少。治疗以强效局部和全身糖皮质激素为主，近期研究强调 doxycycline、dapsone 和 immunosuppressants 的疗效和安全性，旨在降低类固醇的使用量。
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.
