Epidermolysis bullosa dystrophica dominans (Pasini)?A primary structural defect of the anchoring fibrils

Abstract

In epidermolysis bullosa dystrophica dominans Pasini a structural defect of the anchoring fibrils, a structural protein of the epidermo-dermal junction, has been demonstrated by electron microscopy to be constantly present not only in involved skin but also in intact skin of nonpredilection sites of blister formation. These findings render support to the concept that in dominant disorders an abnormality in a non-enzymic structural protein is more likely than in an enzymic protein.