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As vulnerable as a butterfly – New drugs against epidermolysis bullosa

The Eva Luise Köhler Research Award has been awarded annually since 2008 to promote research into rare diseases. The prize money of € 50,000 has already enabled more than a dozen innovative research projects to be launched. The 2009 prizewinner, Prof. Dr. Leena Bruckner-Tuderman, told journalist Sandra Arens how her research in the field of epidermolysis bullosa has developed since receiving the award in 2009.

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Even a hug can be dangerous – just like playing in the garden or riding a bike. Patients with a severe form of the skin disease epidermolysis bullosa have such fragile skin that even the slightest irritation caused by movement and friction can lead to painful blisters and wounds. In Germany, between 1000 and 2000 people are affected.

The disease

The disease group epidermolysis bullosa (EB) is often also called butterfly disease because the skin of patients is as vulnerable as a butterfly’s wing due to a genetic defect. The disease usually manifests itself immediately after birth or shortly thereafter. Depending on which form of EB a child suffers from, the skin as well as mucous membranes and internal organs are affected. Some forms of EB lead to scarring and severe inflammation – sometimes so severe that the fingers grow together or the oesophagus narrows. A carefree life is then no longer possible. In extreme cases, EB can lead to death. There is currently no cure. The therapy consists of avoiding injuries through careful behavior and treating the wounds well.

The research

The scientist and dermatologist Leena Bruckner-Tuderman from the University Dermatology Clinic in Freiburg received the Eva Luise Köhler Research Award in 2009 for her scientific work in the field of epidermolysis bullosa. Together with her team, she investigated many different EB subtypes. “We characterized the molecules in the skin that are altered by the genetic defect and were thus able to discover the causes of the different manifestations of the disease.” Based on these discoveries, Bruckner-Tuderman and her team were able to test new drugs that inhibit scarring in animal experiments and clinical trials – with success: for example, a globally established blood pressure medication works very well against scarring, improving the quality of life of patients.

The future

Leena Bruckner-Tuderman and her research team are currently in contact with a pharmaceutical company in order to test newly developed drugs to alleviate the symptoms in clinical trials based on their scientific findings. “Gene therapies are also being developed in America,” explains the scientist. However, these do not work optimally for severely scarred skin. Bruckner-Tuderman sees the future of promising treatment for EB patients in the combination of anti-scarring medication and subsequent gene therapy. “We hope that patients will be able to benefit from this type of treatment in a few years’ time.”

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