WOUND HEALING
The nature of EB is that the skin blisters at the slightest trauma resulting in wounds that can be very difficult to heal and/or heal with scarring. Wound care and wound healing are, are therefore, crucial aspects of any attempt to alleviate the condition.
On a practical, day-to-day basis, specialist clinicians (many DebRA funded) undertake a great deal of work to improve wound healing. Much of DebRA’s genetic research, which is aimed at a fundamental change in the way the skin adheres, is of great relevance to wound healing.
In addition, the following specific wound healing research is funded.
Details of the following projects can be found on the DebRA International Website, under Current research funded
Developing therapies to control wound healing in EB. Prof. Irwin McLean, University of Dundee Prof. McLean’s group discovered the genetic defect that causes a condition known as LOC syndrome- a specific form of junctional EB. In LOC syndrome patients have a chronic defect in wound healing – they continually produce a type of abnormal tissue, called granulation tissue, in their skin, eyes, mouth, throat and other sites. Granulation tissue is also a problem in other types of EB and in more common conditions, including rheumatoid arthritis, leg ulcers and so on. There is no effective drug at the present time specifically to treat granulation tissue in any of these conditions.
Most LOC patients are lacking a small piece of protein found in the skin known as laminin alpha-3a. Complete loss of laminin alpha-3a causes the severe Herlitz type of junctional EB and loss of this small part of the protein causes LOC. Recently, the group identified one LOC patient with a different type of mutation also affecting this same small part of the laminin alpha-3a protein. This is strong evidence that this region of the laminin alpha-3a protein, which is called the N domain, is a key signal that turns off granulation tissue production at the end of the wound healing process. In LOC patients this signal is absent and so granulation tissue continues to be made. This discovery is very exciting because it tells us for the first time how granulation tissue production is turned off in the skin after a wound is healed and it also opens the way to develop drugs to accelerate this process. This project builds on the discovery towards identifying such drugs.
A novel treatment for wound healing in RDEB. Dr Abhay Pandit, National University of Ireland, Galway. (Funded by DEBRA Ireland.)
The specific objective of this project is to treat patients with debilitating wounds by bridging the gap between basic science on wound healing and the potential clinical application for new technologies.
The central idea of this work is on engineering “smart templates” that guide the regeneration of tissue. This “smart template” will be capable of sensing the environment that it is in and taking necessary actions. The rationale of the project is based on an understanding of the body’s natural processes and incorporates the use of natural biodegradable materials and the signals that the human body deciphers. Hence, the goal of this work is to provide, as part of a skin substitute, a dermal precursor that is capable of directing true skin regeneration.
