Abstract
Autoimmune bullous dermatoses (AIBD) encompass a variety of organ-specific autoimmune diseases that manifest with cutaneous and/or mucosal blisters and erosions. They are characterized by autoantibodies targeting structural proteins of the skin, which are responsible for the intercellular contact between epidermal keratinocytes and for adhesion of the basal keratinocytes to the dermis. The autoantibodies disrupt the adhesive functions, leading to splitting and blister formation. In pemphigus diseases, blisters form intraepidermally, whereas in all other disease types they occur subepidermally. Early identification of autoimmune bullous dermatoses is crucial for both treatment and prognosis, particularly as regards tumor-associated disease entities. The diagnosis is based on clinical symptoms, histopathology, direct immunofluorescence to detect antibody/complement deposits, and the determination of circulating autoantibodies. The identification of various target antigens has paved the way for the recent development of numerous specific autoantibody tests. In particular, optimized designer antigens and multiplex test formats for indirect immunofluorescence and ELISA have enhanced and refined the laboratory analysis, enabling highly efficient serodiagnosis and follow-up. This review elaborates on the current standards in the serological diagnostics for autoimmune bullous dermatoses.
Highlights
Autoimmune bullous dermatoses (AIBD) are associated with autoantibodies that bind to structural proteins in the skin and mucous membranes, which are components of desmosomes and hemidesmosomes (e.g., BP180, BP230, plectin, α6β4 integrin, laminin 332, laminin γ1, type VII collagen) [Figure 1, [1]]
Tissue transglutaminase, endomysium, deamidated gliadin aMain target antigens are indicated in bold. bParameters for which commercial monospecific detection assays are not available are indicated in italics. cImmunodominant regions: Dsg1: N-terminal ectodomain, Dsg3: N-terminal ectodomain, BP180: *NC16A/**LABD97/***linear IgA disease antigen 1 (LAD-1)/****C-terminal epitopes; BP230: globular C-terminal domain; type VII collagen: N-terminal NC1 domain
The serological diagnosis of AIBD follows a multi-step approach that is based on initial indirect immunofluorescence (IIF) screening using one or two tissue substrates, followed by individual antigen-specific assays (ELISA, immunoblot) that correspond to the clinical suspicion and the IIF screening results
Summary
Sandra Saschenbrecker*, Ingolf Karl, Lars Komorowski, Christian Probst, Cornelia Dähnrich, Kai Fechner, Winfried Stöcker and Wolfgang Schlumberger. Autoimmune bullous dermatoses (AIBD) encompass a variety of organ-specific autoimmune diseases that manifest with cutaneous and/or mucosal blisters and erosions. They are characterized by autoantibodies targeting structural proteins of the skin, which are responsible for the intercellular contact between epidermal keratinocytes and for adhesion of the basal keratinocytes to the dermis. The autoantibodies disrupt the adhesive functions, leading to splitting and blister formation. Identification of autoimmune bullous dermatoses is crucial for both treatment and prognosis, as regards tumor-associated disease entities. The identification of various target antigens has paved the way for the recent development of numerous specific autoantibody tests. This review elaborates on the current standards in the serological diagnostics for autoimmune bullous dermatoses
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