EB Research & Resources
What is EB?
Epidermolysis Bullosa (EB) encompasses a group of at least 12 seperate genetic diseases of the skin and sometimes the mucous membranes. It is characterised by skin fragility with blister formation occuring spontaneously or following minor trauma. EB can be broadly divided into three major catagories that are recognised accordingly: Simplex, where cell lysis occurs in the epidermis; Junctional, where the separation occurs within the dermal-epidermal junction, and Dystophic, in which the plan of cleavage is below the basement membrane in the dermis. These catagories can be further subtyped based on inheritance and clinical features.
The ‘Minnesota’ bone-marrow transplantation clinical trial in people with RDEB: the early results
The long-awaited early results of the first clinical trial of bone-marrow transplant in children with recessive dystrophic epidermolysis bullosa (RDEB), carried out at the University of Minnesota in the USA, have now been published in the scientific press. Click here to read the full report.
News from St George Hospital, Sydney
American Academy of Dermatology Conference
This year has been very busy so far for the team at St George Hospital. In March, Professor Dedee Murrell and the team went to Miami for the annual American Academy of Dermatology Conference. This conference is one of the biggest in the world and dermatologists come from every country to hear about new research. Drs Supriya Venugopal and Lizbeth Intong were both selected to present their most recent research at the Residents and Fellow’s symposium at this Conference.
Dermatologic Clinics dedicated to EB
This year the Dermatologic Clinics had two issues dedicated to EB which were edited by Professor Murrell. These issues had contributions from dermatologists worldwide and included Professor Murrell, Lizbeth Intong and Supriya Venugopal.
In Christchurch, New Zealand, the team presented two EB presentations. In addition to this, Dr Heather Cohn won the America-Australia Association Fellowship to perform research with Professor Murrell. Her research looks into genetic risk factors for squamous cell carcinomas in patients with EB.
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Lizbeth Intong, Anna Hanna, Prof Murrell, Supriya Venugopal and Benjamin Daniel at the AAD conference in Miami
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Debra table at the Australasian College of Dermatologists Annual Scientific Meeting in Darwin
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Australasian College of Dermatologists Annual Scientific Meeting
In May, the Australasian College of Dermatologists held their annual Scientific Meeting in Darwin. DebRA had a table at the meeting and was able to provide information to attendees at the conference. Mr Yong Kho, who is a final year medical student and previous ILP student with Professor Murrell, presented the findings from the Australasian Epidermolysis Bullosa Registry in the plenary session. Dr Supriya Venugopal also presented her research on the Investigation of cell therapy for generalised severe dystrophic epidermolysis bullosa by intradermal allogeneic fibrobalsts at the registrar’s forum.
Congratulations Yong!
Congratulations to Yong who had his EB registry paper published in the June Edition of the esteemed journal, Archives of Dermatology.
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And congratulations to Heather Cohn!
Our postdoc, Heather Cohn, has received a prestigious award from the Society for Investigative Dermatology and European Society for Dermatological Research (ESDR) to pay for her to go the ESDR meeting in Helsinki, Finland in Sept to present our cell therapy trial!!
South Australian Research News
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Our Wound Healing Laboratory in Adelaide is led by Prof Allison Cowin and consists of 12 members (both staff and students). We were the first research group to identify Flightless (Flii) protein as a negative regulator of wound healing and were able to show that decreasing the levels of this harmful protein improves wound healing outcomes.
For 7 years now we have been working on developing new therapy for improved wound healing. Majority of our team works on the main project of “Flightless therapy development for treatment of wounds” and this would also include treatment of EB patients. We are excited that this project has now moved from small to large animal models and has entered the pre-clinical stages. This gives us hope that in the next few years we will be able to progress to the human clinical trials, which would involve testing the therapy effectiveness on EB patients.
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In order to better apply this therapy to EB patients our second main EB project is aimed at investigating the role of Flii protein in EB. I am close to submitting my PhD thesis and have now started employed work working together with Research Assistant Mrs Xanthe Strudwick on this project. We have shown that this harmful protein is naturally increased in skin of EB patients so reducing its levels could be expected to improve their healing. Our studies have now shown that this protein is a key factor involved in blister formation and reducing its levels results in reduced blister formation. This gives us hope that our therapy may reduce blister severity and enhance the quality and prolong the life of EB patients.
Also we have started two more EB projects in our laboratory, one looking at the role of Flightless protein in RDEB and SCC and second project looking at its function in the fourth EB subtype - Kindler Syndrome. All our projects are in collaborations with both interstate and overseas collaborators and we are grateful for their help and advice. This is an exciting time for EB research in Adelaide so “WATCH THIS SPACE” for more updates on our research and therapy outcomes for the future.
Kind regards
Dr Zlatko Kopecki
Women’s and Children’s Health Research Institute
Wound Healing Laboratory, Adelaide
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Above: Prof Allison Cowin
Below: Some of our Wound Healing laboratory staff
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EB Resources & Downloads
Summary of Epidermolysis Bullosa Subtypes and Genetics Download
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