Abstract
Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous dermatosis characterized by chronic mucocutaneous blistering caused by autoantibodies directed against type VII collagen. EBA causes a high morbidity and is difficult to treat. Model systems have significantly broadened our understanding of EBA pathogenesis, leading to the identification of numerous therapeutic targets. Of these, so far, a few have been evaluated for their therapeutic potential in preclinical models. In mice, EBA can be induced by transfer of anti-type VII collagen antibodies or by immunization with the protein. The latter model, immunization-induced EBA, is ideal to test drugs for their therapeutic efficacy. Here, mice with already established disease can be treated for prolonged periods. Albeit time consuming, results from immunization-induced EBA will pave the way for clinical application in patients. As the key pathogenic principle, that is, autoantibody-induced, leukocyte-mediated tissue injury and inflammation, is shared by other diseases, these findings may have translational applications beyond EBA.
| Original language | English |
|---|---|
| Journal | Expert Review of Clinical Immunology |
| Volume | 11 |
| Issue number | 12 |
| Pages (from-to) | 1365-1378 |
| Number of pages | 14 |
| ISSN | 1744-666X |
| DOIs | |
| Publication status | Published - 02.12.2015 |
Funding
This research has been supported by funding from the Deutsche Forschungsgemeinschaft as well as research grants from Dompe, Biotest and Suppremol. The author received support for research and/or honoraria from the following companies: Dompe, Biotest AG and Suppremol, which produce/market drugs discussed in this review. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.
