Bullous pemphigoid

被引:170
作者
Bagci, Isin Sinem [1 ,2 ]
Horvath, Orsolya N. [1 ]
Ruzicka, Thomas [1 ]
Sardy, Miklos [1 ,3 ]
机构
[1] Ludwig Maximilians Univ Munchen, Dept Dermatol & Allergol, Frauenlobstr 9-11, D-80337 Munich, Germany
[2] Ankara 29 Mayis Govt Hosp, Dept Dermatol, Ankara, Turkey
[3] Semmelweis Univ, Dept Dermatol Venereol & Dermatooncol, Budapest, Hungary
关键词
Bullous pemphigoid; Autoimmune bullous diseases; Pemphigoid diseases; LOW-DOSE METHOTREXATE; BRUNSTING-PERRY TYPE; AUTOREACTIVE T-CELL; BLISTER FORMATION; AUTOIMMUNE-DISEASE; XVII COLLAGEN; RISK-FACTORS; TOPICAL CORTICOSTEROIDS; RECOMBINANT PROTEINS; AUTOANTIBODY PROFILE;
D O I
10.1016/j.autrev.2017.03.010
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
071005 [微生物学]; 100108 [医学免疫学];
摘要
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. (C) 2017 Elsevier B.V. All rights reserved.
引用
收藏
页码:445 / 455
页数:11
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