D'Agostino Giovanni Marco, Rizzetto Giulio, Marani Andrea, Marasca Samuele, Candelora Matteo, Gambini Daisy, Gioacchini Helena, De Simoni Edoardo, Maurizi Andrea, Campanati Anna, Offidani Annamaria
Dermatological Clinic, Department of Clinical and Molecular Sciences, Polytechnic Marche University, 60126 Ancona, Italy.
Biomedicines. 2022 Nov 8;10(11):2844. doi: 10.3390/biomedicines10112844.
Bullous pemphigoid is a subepidermal blistering disease associated with autoantibodies (auto-ab) to BP180 and BP230 which affects elderly patients, predominately. Although it is a rare disease, bullous pemphigoid is the most common among the autoimmune bullous skin diseases. Systemic corticosteroids and immunosuppressants represent milestones in the treatment of patients suffering from bullous pemphigoid; however, therapeutic management of patients still represents a clinical challenge, owing to the chronic nature of the disease and to potential adverse effects related to the long-term use of systemic treatments. Recent discoveries on the pathogenesis of bullous pemphigoid have allowed investigation of new target therapies against selective pro-inflammatory mediators. These therapies appear to yield satisfactory results with fewer side effects in cases of refractory disease. The review discusses current evidence on these new therapeutic targets and specific drugs under investigation.
大疱性类天疱疮是一种与针对BP180和BP230的自身抗体相关的表皮下大疱性疾病，主要影响老年患者。尽管它是一种罕见疾病，但大疱性类天疱疮是自身免疫性大疱性皮肤病中最常见的。系统性糖皮质激素和免疫抑制剂是治疗大疱性类天疱疮患者的里程碑；然而，由于该疾病的慢性性质以及与长期使用系统性治疗相关的潜在不良反应，患者的治疗管理仍然是一项临床挑战。关于大疱性类天疱疮发病机制的最新发现使得针对选择性促炎介质的新靶向治疗得以研究。这些疗法在难治性疾病的情况下似乎能产生令人满意的结果，且副作用较少。本文综述讨论了这些新治疗靶点和正在研究的特定药物的当前证据。
