The pathophysiology of autoimmune blistering diseases
J. Clin. Invest. Kim B. Yancey, et al. 115:825 doi:10.1172/JCI24855 [Go to this article.]

Figure 2
Schematic overview of the plasminogen/plasmin activation cascade in experimental murine BP. Anti-BP180 IgG binds epidermal BM, activates complement, and generates neutrophil-rich infiltrates in skin. Subsequently, plasmin activates MMP-9, which in turn inactivates α1–proteinase inhibitor (α1-PI), thus allowing unrestrained activity of neutrophil elastase that degrades BP180 and produces subepidermal blisters. tPA, tissue plasminogen activator; uPA, urokinase plasminogen activator.