Epidermolysis Bullosa

The Epidermolysis Bullosa (EB) is a rare genetic disease that affects the skin and the deep mucous membranes. Because of hereditary factors, the skin forms blisters and bubbles and tends to tear off with great pain as a result of minor trauma or friction , or even spontaneously. For this reason, children with this rare disease are called "butterfly children" because their skin is fragile as the wings of butterflies. Living with EB has been compared to living with a third degree burn. It is a painful condition that requires to the parents an immediate extreme care and the adoption of special precautions in child handling and nutrition. Sufferers must undergo every day, even for hours, to medications and bandages to protect wounds and antibiotic therapies are often required to counteract the onset of infections. In more severe case , surgery may be needed for the expansion of the esophagus and the reconstruction of the hands and there is also the risk of skin cancer and complications to the kidneys .
The EB has also a major impact on the psychological and social life , not only because it is a "visible" disease that causes the curiosity of others but also because it makes it very difficult , sometimes impossible , natural gestures such as running, playing , hugging and be embraced.

The EB is a rare disease (in Italy, affecting 1 every 82,000 births) and, as often happens, it is also a "orphan" disease, meaning that its small numbers attracts few research investments.
There are three major forms of EB (Simplex, Dystrophic and Junctional), each of which includes other subspecies and each case may present with symptoms vary widely, from mild to very severe and even fatal. This is because it is a genetic disorder caused by hereditary factors that can be dominant (transmission from a sick parent) or recessive (transmission from both carrier parents) and therefore involve different genes .
In 50% of cases, the EB occurs in the form Simplex, generally less severe, involving mainly the hands and feet.

The choice of clothing is crucial for people with EB and a particular attention must be give especially to underwear, which is in direct contact with the skin and must be particularly soft and fresh and limit as much as possible frictions.

DermaSilk therapeutic clothing, specially designed for those suffering from diseases of the skin and mucous membranes, has proven very useful and effective in Epidermolysis Bullosa, reducing the symptoms and improving significantly the quality of life of patients.

For this reason, following positive clinical evaluations and the active involvement of DEBRA Italy Onlus (Association of Patients with EB ), from 2010, in Italy, people with EB are entitled to the reimbursement of DermaSilk products by the National Health Service.

Thanks to its characteristics, DermaSilk therapeutic clothing in knitted pure silk fibroin with permanent antimicrobial protection is able to:

  • reduce mechanical friction and protects the skin;
  • reduce itching and inflammation;control and prevent superinfection by bacteria, fungi and yeasts;
  • control perspiration and maintain the proper moisture balance;
  • promote wound healing and skin regeneration.

The effectiveness of DermaSilk in reducing symptoms and improving the quality of life of patients with EB has been highlighted by a study conducted at the EB -Haus Austria - Salzburg Hospital.