Epidermolysis bullosa (EB)
EB is a very rare genetic skin condition affecting only 8.22 new cases per one million births worldwide. The hallmarks of EB are skin fragility and blisters formation at the sites of even the mildest mechanical trauma. Younger patients are hence often called ‘Butterfly children’ due to the fact that their skin is as fragile as butterfly’s wings. People affected by any type of EB experience extensive blistering and wounding all over the body which in a more severe form can also include the mucosa membranes of the oral tract and internal organs. Another challenging symptom of EB is intensive itch. Such provoke patients to scratch, which in result expand cutaneous damage, often leading to chronically inflamed and slow to non-healing skin wounds. This increases the risk of the development of squamous cell carcinoma (SCC), an aggressive type of skin cancer.
At present, there is still no existing cure for EB. The current standard of care focuses on improvement of patient’s quality of life by skin protection, wound healing promotion, pain management and nutritional support. However, the presence of multiple wounds of differing characteristics makes handling of EB difficult and complex. Especially challenging is oral care management due to the fact that there are no ointments or dressings designated for mucosa wounds. The dental treatment bases only on palliative care with typical restorative and periodontal procedures. The alleviatory care intends to reduce symptoms mostly by repeated use of steroids (e.g. Cortisone) which are know for their side effects in case of long term use. Importantly, also analgesic medications may lead to various unwanted reactions and intensify symptoms of EB (e.g. pruritus).
Due to the fragile nature of EB skin, improvement of wound care and pain management is of uppermost medical need.
About liposomal rhSOD (SCR201)
Superoxide Dismutase (SOD) is nature’s most efficient anti-oxidative enzyme acting by neutralizing harmful superoxide anion radicals. The liposomal formulation of recombinant human SOD (SCR201) has excellent anti-oxidant and anti-inflammatory properties. Topical format of SCR201 reached already a highly advanced clinical developmental stage. Previous trials have proven its safety and revealed its high potential for the treatment of skin diseases marked by pronounced epidermal barrier defect. In many pre-clinical and clinical trials rhSOD has been demonstrated to accelerate healing process of acute and chronic wounds and remarkably lower pain sensation without any adverse effects. Moreover, SOD was demonstrated to diminish itchiness, which is one of the main factors lowering quality of life in patients with EB.
CEO, Schnidar: Combined treatment of EB could reduce the use of analgesic medications likewise steroids. At present, none of the dressings recommended and tested in EB wound care is characterized by such broad spectrum of drug action. In addition to this, clinical development of rhSOD may provide the first treatment possibility of mucosae wounds in oral cavity in EB.