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June 1982

Epidermolysis Bullosa-Reply

Arch Dermatol. 1982;118(6):372. doi:10.1001/archderm.1982.01650180005006

In Reply.—  We thank Drs Ayres and Mihan for their comments on our editorial. We want to emphasize, however, that we were discussing only recessive dystrophic epidermolysis bullosa (RDEB), not the simplex or other forms of the disease. For RDEB, there are both in vivo1-3 and in vitro4-8 data from several laboratories that strongly suggest that collagenase plays some role in the pathogenesis of the disease. This enzyme is synthesized in increased quantities both in explant cultures4,5 and in fibroblast cultures.7,8 Indeed, RDEB fibroblasts, but no other types, in cell culture express the increased synthesis of a structurally aberrant collagenase,9 suggesting that the biochemical trait is genetically determined.7 On the basis of current knowledge, we find it difficult to ascribe this persistently high synthetic rate to lysosomal destruction. Furthermore, except for granulocyte collagenase, there is no evidence to support the assertion that collagenase is