Department of Dermatology, Inselspital, Bern University Hospital, Bern, Switzerland.
Department of Dermatology, University of Lübeck, Lübeck, Germany.
J Eur Acad Dermatol Venereol. 2022 Oct;36(10):1689-1704. doi: 10.1111/jdv.18220. Epub 2022 Jun 29.
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life.
The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included.
Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations.
The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.
大疱性类天疱疮（BP）是皮肤和黏膜最常见的自身免疫性表皮下水疱病。这种疾病通常发生在老年人身上，表现为瘙痒和局部或最常见的全身性大疱性病变。一部分患者仅出现搔抓、痒疹样病变、湿疹样和/或荨麻疹样红斑。该疾病与神经障碍显著相关，发病率高，严重影响生活质量。
欧洲皮肤病学会和性病学会的自身免疫性水疱病工作组旨在根据新的临床信息以及新的诊断工具和干预措施的证据，更新 BP 的管理指南。建议要么基于证据，要么依赖专家意见。还包括了所有工作组成员的一致程度。
治疗取决于 BP 的严重程度和患者的合并症。只要可能，建议使用强效局部皮质类固醇作为主要治疗方法。推荐口服泼尼松 0.5mg/kg/天作为替代治疗。如果存在皮质类固醇的禁忌症或耐药性，可推荐免疫抑制治疗，如甲氨蝶呤、硫唑嘌呤、霉酚酸酯或霉酚酸。多西环素和氨苯砜的使用存在争议。特别是在对口服皮质类固醇有禁忌症的患者中，可能会推荐使用。在治疗抵抗的情况下，可以考虑 B 细胞耗竭疗法和静脉注射免疫球蛋白。奥马珠单抗和度普利尤单抗最近显示出了有前途的结果。该指南的最终版本得到了多个患者组织的认可。
更新了 BP 管理指南。它们总结了在临床实践中有用的基于证据和专家意见的建议。
