Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Centre for Biological Signaling Studies (BIOSS), University of Freiburg, Freiburg, Germany.
Australas J Dermatol. 2022 May;63(2):165-171. doi: 10.1111/ajd.13808. Epub 2022 Feb 23.
Lichen planus pemphigoides (LPP) and bullous lichen planus (BLP) are rare dermatoses, which are characterised by blisters and lichenoid lesions. Their clinical presentation is heterogenous, displaying overlapping features or mimicking other dermatological diseases. Therefore, diagnosis can often be challenging, requiring a thorough dermatological examination along with distinctive histological and immunopathological characteristics. Lichenoid degeneration of the basal epidermis exposes various antigens of the dermal-epidermal junction in LPP, resulting in the breakdown of immune tolerance, hence, the production of autoantibodies against type XVII collagen. Conversely, no pathogenic autoantibodies are detected in BLP. However, some cases of mucosal lichen planus might display immunopathological features suggestive of autoimmune blistering diseases. Therefore, a better understanding of the pathophysiology of these two distinct dermatoses is imperative. The aim of this review was to provide a summary of the current knowledge on the clinical hallmarks, diagnosis and available therapeutic options in LPP and BLP.
天疱疮样扁平苔藓（LPP）和大疱性扁平苔藓（BLP）是两种罕见的皮肤疾病，其特征为水疱和苔藓样损害。它们的临床表现具有异质性，存在重叠特征或模仿其他皮肤疾病。因此，诊断通常具有挑战性，需要进行彻底的皮肤科检查，同时具备独特的组织学和免疫病理学特征。LPP 中基底表皮的苔藓样变性使表皮-真皮交界处的各种抗原暴露，导致免疫耐受的破坏，从而产生针对 XVII 型胶原的自身抗体。相反，BLP 中未检测到致病性自身抗体。然而，一些黏膜扁平苔藓病例可能表现出免疫病理学特征，提示自身免疫性水疱病。因此，更好地了解这两种不同的皮肤病的病理生理学至关重要。本文旨在综述 LPP 和 BLP 的临床特征、诊断和现有治疗选择的相关知识。
