Injury- and inflammation-driven skin fibrosis: The paradigm of epidermolysis bullosa.

Abstract

Genetic or acquired destabilization of the dermal extracellular matrix evokes injury- and inflammation-driven progressive soft tissue fibrosis. Dystrophic epidermolysis bullosa (DEB), a heritable human skin fragility disorder, is a paradigmatic disease to investigate these processes. Studies of DEB have generated abundant new information on cellular and molecular mechanisms at play in skin fibrosis which are not only limited to intractable diseases, but also applicable to some of the most common acquired conditions. Here, we discuss recent advances in understanding the biological and mechanical mechanisms driving the dermal fibrosis in DEB. Much of this progress is owed to the implementation of cell and tissue omics studies, which we pay special attention to. Based on the novel findings and increased understanding of the disease mechanisms in DEB, translational aspects and future therapeutic perspectives are emerging.

• Authors: Alexander Nyström, Leena Bruckner-Tuderman
• Journal: Matrix biology : journal of the International Society for Matrix Biology (Matrix Biol) Vol. 68-69 Pg. 547-560 (Aug 2018) ISSN: 1569-1802 [Electronic] Netherlands
• PMID: 29391280 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2018 Elsevier B.V. All rights reserved.

Chemical References

• COL7A1 protein, human
• Collagen Type VII

Topics

• Animals
• Collagen Type VII (genetics)
• Disease Models, Animal
• Epidermolysis Bullosa Dystrophica (genetics, metabolism, pathology)
• Extracellular Matrix (genetics)
• Gene Expression Profiling
• Gene Knockout Techniques
• Humans
• Mice
• Mice, Transgenic