Heckler Ilana, Hong Michael, Amart Sinha Animesh, Venkataraman Iswariya
EUROIMMUN US Inc, 1 Bloomfield Ave, Mountain Lakes, New Jersey, United States.
College of Medicine, Florida State University, Tallahassee, Florida, United States.
Dermatol Pract Concept. 2022 Apr 1;12(2):e2022116. doi: 10.5826/dpc.1202a116. eCollection 2022 May.
Autoimmune bullous diseases (AIBDs) are a group of skin-related disorders that involve damage to structures maintaining cell-cell adhesion, such as desmosomes and hemidesmosomes. Key AIBDs include pemphigus related diseases, pemphigoid related conditions, acquired epidermolysis bullosa (EBA), and dermatitis herpetiformis (DH). Each group of conditions exhibits characteristic clinical lesion patterns and is associated with specific autoantibodies targeting epidermal and dermal structures involved in cell-cell adhesion and skin integrity. Pemphigus diseases primarily target desmoglein (Dsg) 3 and Dsg1 proteins but several non-Dsg autoantibodies have also been linked to pemphigus. Pemphigoid diseases typically target bullous pemphigoid (BP)180 and BP230; EBA is associated with antibodies directed against anti-type VII collagen and DH by IgA autoantibodies against tissue transglutaminase and deaminated gliadin. Investigation into the serological biomarkers found in AIBDs have allowed the development of diagnostic assessments (i.e. tissue antibody detection and serological testing) based on the unique autoantibody profiles of a particular disease group. The methods for the detection and quantification of disease-associated autoantibodies continue to evolve and improve.
自身免疫性大疱性疾病(AIBDs)是一组与皮肤相关的疾病,涉及对维持细胞间粘附的结构(如桥粒和半桥粒)的损害。主要的AIBDs包括天疱疮相关疾病、类天疱疮相关病症、获得性大疱性表皮松解症(EBA)和疱疹样皮炎(DH)。每组病症都表现出特征性的临床病变模式,并与针对参与细胞间粘附和皮肤完整性的表皮和真皮结构的特定自身抗体相关。天疱疮疾病主要靶向桥粒芯糖蛋白(Dsg)3和Dsg1蛋白,但也有几种非Dsg自身抗体与天疱疮有关。类天疱疮疾病通常靶向大疱性类天疱疮(BP)180和BP230;EBA与针对抗VII型胶原蛋白的抗体相关,而DH则与针对组织转谷氨酰胺酶和脱氨麦醇溶蛋白的IgA自身抗体相关。对AIBDs中发现的血清生物标志物的研究使得基于特定疾病组独特的自身抗体谱开发出诊断评估方法(即组织抗体检测和血清学检测)。疾病相关自身抗体的检测和定量方法不断发展和改进。