Cozzani Emanuele, Marzano Angelo V, Caproni Marzia, Feliciani Claudio, Calzavara-Pinton Piergiacomo
Clinic of Dermatology, University of Genoa, Genoa, Italy.
Unit of Dermatology, Department of Pathophysiology, Surgery, and Transplantation, University of Milan, Ca' Granda Foundation and Institute for Research and Care, Maggiore Polyclinic Hospital, Milan, Italy.
G Ital Dermatol Venereol. 2018 Jun;153(3):305-315. doi: 10.23736/S0392-0488.18.06006-6. Epub 2018 Mar 30.
Bullous pemphigoid 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 generalized bullous lesions. In up to 20% of affected patients blister may be completely absent, and only excoriations, prurigo-like lesions, eczematous lesions, urticated lesions, and/or infiltrated plaques are observed. The disease is significantly associated with neurological disorders. The morbidity of bullous pemphigoid and its impact on the quality of life are significant. So far, a limited number of national treatment guidelines have been proposed, but no common European consensus has emerged. This guideline for the treatment of bullous pemphigoid has been developed by an Italian group of experts taking in account the Italian legislation and local pharmacological governance. Guidelines are adapted from the original article under the guidance of the European Dermatology Forum (EDF) in collaboration with the European Academy of Dermatology and Venereology (EADV). It summarizes evidence-based and expert-based recommendations (S2 level).
大疱性类天疱疮是皮肤和黏膜最常见的自身免疫性表皮下大疱性疾病。该病通常累及老年人，表现为瘙痒及局限性或全身性大疱性皮损。高达20%的受累患者可能完全没有水疱，仅观察到抓痕、痒疹样皮损、湿疹样皮损、风团样皮损和/或浸润性斑块。该病与神经系统疾病显著相关。大疱性类天疱疮的发病率及其对生活质量的影响都很显著。到目前为止，仅提出了有限数量的国家治疗指南，但尚未形成欧洲共识。本大疱性类天疱疮治疗指南由一组意大利专家制定，考虑了意大利的立法和当地的药理学管理。指南在欧洲皮肤病学论坛（EDF）与欧洲皮肤病与性病学会（EADV）的合作指导下，改编自原文。它总结了基于证据和专家的建议（S2级）。
