自身免疫性起泡疾病管理可能具有挑战性,因为治疗方式差异很大,并且没有单一的护理标准。我们合并了国际管理指南的建议,以便为医生提供最佳的管理建议。2019年11月在PubMed/MEDLINE中进行了全面的文献检索,以了解已发布的水疱疾病管理指南和共识声明。搜索术语包括“指南或指南”或“共识”和“类天疱疮”或“自身免疫性水疱病”或“大疱性表皮松解症”。我们纳入了已建立的皮肤病学会和专家共识小组的指南。我们排除了文献综述,由没有皮肤科医生的协会制定的指南,或特定于单一治疗的那些。考虑了所有语言的准则。选择了符合我们纳入标准的皮肤病学协会和共识组的11个指南。建议之间的几个差异,最值得注意的是什么时候为难治性疾病引入佐剂,在大疱性类天疱疮中发现。在粘膜类天疱疮中,治疗针对所涉及的部位,并使用全身性皮质类固醇和免疫抑制剂/生物制剂进行管理.对于大疱性表皮松解症的一线治疗尚无普遍共识,但是免疫抑制的组合,抗炎,使用抗中性粒细胞治疗。管理指南的比较显示,发展中国家的指南代表性不足,欧洲和亚洲的皮肤科医生之间的管理方式存在主要差异。我们将这些差异归因于指南之间经过的时间,地区差异,以及当地医疗系统的需求。
Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the recommendations of international management guidelines in order to provide optimal management suggestions to physicians. A comprehensive literature search in PubMed/MEDLINE for published blistering disease management guidelines and
consensus statements was conducted in November 2019. Search terms included \"
guideline or
guidelines\" or \"
consensus\" and \"pemphigoid\" or \"autoimmune blistering disease\" or \"epidermolysis bullosa acquisita\". We included
guidelines from established dermatologic societies and expert
consensus groups. We excluded literature reviews, guidelines established by an association without dermatologists, or those specific to a single treatment. Guidelines in all languages were considered. Eleven
guidelines from dermatologic associations and
consensus groups meeting our inclusion criteria were selected. Several differences between recommendations, most notably when to introduce adjuvants for refractory disease, were found in bullous pemphigoid. In mucous membrane pemphigoid, treatment was directed to the sites involved and managed with systemic corticosteroids and immunosuppressants/biologics. There was no universal
consensus on the first-line treatment for epidermolysis bullosa acquisita, but a combination of immunosuppressive, anti-inflammatory, and anti-neutrophil therapy was utilized. Comparison of the management guidelines revealed underrepresentation of guidelines from developing nations and key differences between the management styles among dermatologists from Europe and Asia. We attribute these discrepancies to the time elapsed between guidelines, regional differences, and demands of the local healthcare systems.