Abstract
Autoimmune skin blistering diseases (AIBD) are characterized by autoantibodies that are directed against structural proteins in the skin and adjacent mucous membranes. Some clinical signs are typical for a specific AIBD, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. The gold standard for diagnosis of AIBD is the detection of autoantibodies or complement component 3 by direct immunofluorescence (DIF) microscopy of a perilesional biopsy. Circulating antibodies can be detected via indirect immunofluorescence (IIF) microscopy of different tissue substrates including human skin, monkey esophagus, and more recently, recombinant forms of the different target antigens. Latter are also employed in various commercial ELISA systems and by immunoblotting in in-house assays available in specialized laboratories. ELISA systems are also particularly valuable for monitoring of the disease activity during the disease course which can be helpful for treatment decisions. Exact diagnosis is essential for both treatment and prognosis, since some AIBD are associated with malign tumors such as paraneoplastic pemphigus and anti-laminin 332 mucous membrane pemphigoid. This review presents clinical and immunopathological features of AIBD for the state-of the art diagnosis of these disorders.
🔬 Techniques
🧪 Sample Preparation
🔬 Cell Lines
🏛️ Research Organizations (ROR)
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📊 Figures
Figure 1
Differential diagnosis of autoimmune skin blistering diseases based on direct immunofluorescence (DIF) microscopy of a perilesional biopsy. BP, bullous pemphigoid; EBA, epidermolysis bullosa acquisita...
Figure 2
Clinical and immunopathological characteristics in bullous pemphigoid (BP, left ), pemphigus vulgaris (PV, middle ), and epidermolysis bullosa acquisita (EBA, right ).
Figure 3
n-serrated (left) and u-serrated pattern (right) of basement membrane zone staining in pemphigoid diseases detected by direct immunofluorescence microscopy. While an u-serrated pattern is exclusively ...
Figure 4
Differential diagnosis of autoimmune skin blistering diseases based on the serological detection of autoantibodies. Dsc, desmocollin; Dsg, desmoglein; EBA, epidermolysis bullosa acquisita; * commercia...
Figure 5
Anti-laminin 332 BIOCHIPu00ae mosaic. The mosaic contains HEK293 cells expressing the recombinant laminin 332 heterotrimer (Trim; upper left and middle left), the recombinant u03b32- t u03b23-, and u0...
Figure 6
Detection of serum autoantibodies against the p200 protein and type VII collagen by Western blotting with extract of human dermis. Sera from a patient with anti-p200/laminin u03b31 pemphigoid and epid...
Figure 7
Detection of IgG and IgA autoantibodies against LAD-1 by Western blotting with the concentrated conditioned medium of cultured human keratinocytes. Sera from patients with bullous pemphigoid (BP), muc...
Figure 8
Clinical findings in mucous membrane pemphigoid affecting the conjunctiva, lips, gingiva, and glans penis.
Figure images are served from the NIH/NLM PubMed Central Open Access Subset or Europe PMC; copyright remains with the publishers and authors.
💬 Discussion
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