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大疱性类天疱疮。

Bullous pemphigoid.

机构信息

Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany; Department of Dermatology, Ankara 29 Mayıs Government Hospital, Ankara, Turkey.

Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany.

出版信息

Autoimmun Rev. 2017 May;16(5):445-455. doi: 10.1016/j.autrev.2017.03.010. Epub 2017 Mar 8.

Abstract

Bullous pemphigoid (BP) is the most common autoimmune bullous disorder which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. Collagen XVII and dystonin-e have been identified as target antigens. BP affects mostly the elderly. The incidence of the disease is increasing gradually and is associated with high morbidity and mortality. Clinically, BP is characterized by an intensely pruritic eruption with widespread bullous lesions. The clinical diagnosis can be challenging in the setting of atypical presentations. Diagnosis of BP relies on the integration of clinical, histological, immunopathological, and serological findings. The treatment is mainly based on topical and/or systemic glucocorticoids, but anti-inflammatory antibiotics and steroid sparing adjuvants are useful alternatives. Localised and mild BP can be treated with topical corticosteroids alone.

摘要

大疱性类天疱疮(BP)是最常见的自身免疫性大疱性疾病,其特征是针对皮肤和粘膜的半桥粒蛋白的自身抗体。现已鉴定出胶原 XVII 和 dystonin-e 为靶抗原。BP 主要影响老年人。该疾病的发病率逐渐增加,且与高发病率和死亡率相关。临床上,BP 的特征是剧烈瘙痒伴有广泛的大疱性皮损。在不典型表现的情况下,临床诊断可能具有挑战性。BP 的诊断依赖于临床、组织学、免疫病理学和血清学发现的综合。治疗主要基于局部和/或全身糖皮质激素,但抗炎抗生素和类固醇节省辅助剂是有用的替代方法。局限性和轻度 BP 可以单独用局部皮质类固醇治疗。

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