Decompensation in proteinase-inhibitor system and application of proteinase inhibitors in pemphigus and pemphigoid.

The goal of this study was to determine levels of serine proteinases and their inhibitors in serum and blister fluid of patients with pemphigus vulgaris (PV) and bullous pemphigoid (BP) in the course of treatment with prednisolone and proteinase inhibitors such as Contrycal and epsilon-aminocaproic acid. Assessment of the total proteolytic activity of serine proteinases (TPASP) and total antitryptic activity (TATA) is sera and blister fluids from 53 PV and 38 BP patients showed abnormalities in the proteinase and proteinase inhibitor levels in the acute stage of these bullous dermatoses. In PV, the level of TRASP was raised to 495 +/- 73 mU/ml (control 246 +/- 48 mU/ml; P < 0.001) in serum and to 849 +/- 96 mU/ml (control 189 +/- 34 mU/ml; P < 0.001) in blister fluid. In BP, the level of TRASP was raised to 472 +/- 101 mU/ml (P < 0.001) and 796 +/- 103 mU/ml (P < 0.001) in serum and blister fluid, respectively. In both dermatoses, the increase in serine proteinase activity developed against the background of decreased or unchanged antiproteolytic activity. TATA levels dropped to 16 +/- 5 inhibitory units (iU)/ml (control 26 +/- 5 iU/ml; P < 0.001) in PV serum and to 6 +/- 1 iU/ml (control 20 +/- 2 iU/ml: P < 0.001) in PV blister fluid. In BP, serum TATA levels were within normal values, whereas blister fluid TATA levels were significantly decreased (14 +/- 3 iU/ml; P < 0.1).(ABSTRACT TRUNCATED AT 250 WORDS)