Bullous pemphigoid.

IF 76.9 1区 医学 Q1 MEDICINE, GENERAL & INTERNAL Nature Reviews Disease Primers Pub Date : 2025-02-20 DOI:10.1038/s41572-025-00595-5
Hossein Akbarialiabad, Enno Schmidt, Aikaterini Patsatsi, Yen Loo Lim, Anisa Mosam, Kaisa Tasanen, Jun Yamagami, Maryam Daneshpazhooh, Dipankar De, Adela Rambi G Cardones, Pascal Joly, Dedee F Murrell
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Abstract

Bullous pemphigoid is a chronic, subepidermal autoimmune blistering disease characterized by tense blisters on erythematous or normal skin that predominantly affects the older population. The disease arises from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, which are crucial for dermal-epidermal adhesion. The incidence of bullous pemphigoid is increasing, attributed to an ageing population and improved diagnostic recognition. Genetic predisposition, environmental triggers and associations with other autoimmune disorders underline its multifactorial nature. Diagnosis involves clinical presentation, histopathology, direct immunofluorescence and serological tests. Treatment aims to reduce symptoms and prevent new blister formation, using corticosteroids, immunosuppressive agents and biologics such as rituximab and omalizumab. Despite therapeutic advancements, challenges persist in long-term management, especially in older patients with comorbidities. Ongoing research into molecular mechanisms and novel therapeutic targets and clinical trials are crucial for the development of safer and more effective treatments.

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大疱的类天疱疮。
大疱性类天疱疮是一种慢性,表皮下自身免疫性起泡疾病,其特征是红斑或正常皮肤上的紧张水泡,主要影响老年人。这种疾病是由针对半粒染色体蛋白BP180和BP230的自身抗体引起的,这两种蛋白对真皮-表皮粘附至关重要。由于人口老龄化和诊断认识的提高,大疱性类天疱疮的发病率正在增加。遗传易感性、环境诱因和与其他自身免疫性疾病的关联强调了其多因素性质。诊断包括临床表现、组织病理学、直接免疫荧光和血清学检查。治疗的目的是减轻症状和防止新的水疱形成,使用皮质类固醇、免疫抑制剂和生物制剂,如利妥昔单抗和奥玛珠单抗。尽管治疗取得了进步,但长期管理仍然存在挑战,特别是在有合并症的老年患者中。正在进行的分子机制研究和新的治疗靶点和临床试验对于开发更安全、更有效的治疗方法至关重要。
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来源期刊
Nature Reviews Disease Primers
Nature Reviews Disease Primers Medicine-General Medicine
CiteScore
76.70
自引率
0.20%
发文量
75
期刊介绍: Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.
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