Dystrophic Epidermolysis Bullosa (Spain) Top Priorities (priority setting in association with the JLA)

  1. Which wound care method obtains better outcomes (improved healing, decrease pain, improve quality of life, decrease smell, prevent infection) in patients with EB? Interventions include types of dressings (polyethylene, polyester plus petrolatum, hydrocolloid, collagen, hydrofiber, hydrogel, silicone…), topical antibacterial treatment (clorhexidine, bleach bath, vinegar bath, honey, antibiotics, silver dressings) and frequency of cure (daily or alternate days)? 
  2. What is the best treatment to control itch in DEB patients (sedating antihistaminics, non-sedating antihistaminics, topical menthol, topical corticosteroids, moisturizers, doxepin, gabapentine, cyclosporine, dronabinol, ondansetron)? 
  3. What is the best pain control strategy (analgesics, sedative drugs, addition of NaCl into the water) to decrease pain during wound care and bath in DEB patients? 
  4. How much does management in reference centers help patients with DEB (in terms of quality of life, avoiding complications and disability, cost-effectiveness)? 
  5. How effective is a "tumor early diagnosis protocol" in patients with DEB to decrease mortality, amputations and disability? 
  6. What are the long-term results of syndactyly surgery? Which is the best technique? How often should it be performed? 
  7. Which is the most effective method in avoiding or delaying syndactyly in patients with DEB? Including different types of bandages, dressings, gloves and splints, physiotherapy and occupational therapy. 
  8. What role might tissue engineering have in treating wounds in patients with DEB?
  9. What role might stem cell therapy and bone marrow transplantation play in treating DEB? 
  10. What role might growth hormone play in decreasing growth delay and puberty delayed in DEB patients?