[Vesicular fluid of hereditary bullous dystrophic epidermolysis splits alpha 1-antitrypsin]

In epidermolysis hereditaria bullosa dystrophica increased collagenase activity can be detected and seems to be one of the pathogenetic mechanisms of this disease. Neither the origin nor the mechanism of increased collagenolysis is known. Whether the cause of the enzymatic imbalance is the increased collagenase production or decreased collagenase-inhibitor activity cannot be decided. Factors of decreased protease inhibitor activity could be the quantitative or qualitative defect or the inactivation of the inhibitor. Clear, sterile vesicular fluid was incubated with alpha-1-antitrypsin, which is known to inhibit collagenase. By means of an immunoelectrophoretic method the cleaving of the inhibitor into two antigenic split products was found. We suggest that this might be responsible for the increased collagenolysis in this form of epidermolysis.