What is epidermolysis bullosa (EB disease)?
Epidermolysis bullosa (EB) is a skin disease. The different layers of the skin are not good to each other. Let the skin therefore easy and quickly you will get blisters.
On healthy skin blistering only occurs in severe friction. People with EB disease get all blisters example by wearing clothing. The disease often causes pain, itch and scarring.
There are different forms. At the milder forms are only the blisters on the hands and feet. In the severe forms may the blisters all over the body, also in the mouth and pharynx. This allows serious gaps and growth deformities. Babies with a severe form of EB disease are sometimes only a few weeks old.
No form of epidermolysis bullosa is contagious. The disease is hereditary. There is no treatment against EB disease. The important thing is that the wounds well cared for. This prevents new blisters and wound infections.
Another name is blister disease.
Forms of epidermolysis bullosa (EB disease)
There are three forms of epidermolysis bullosa (EB):
- Epidermolysis bullosa simplex. In most people with this form are only the blisters on the hands and feet. Sometimes they sit all over the body.
- Junctional epidermolysis bullosa. This is form of EB sometimes mild and sometimes is fatal. This form also blisters in the pharynx and the esophagus. For example by ' hard foods like crackers, but also just. At a certain type of junctional EB created large wounds in the face. There may also be accumulations of bright red granular tissue. This is a very serious illness. Babies with the Herlitz-variant are often only a few months old.
- Dystrophic epidermolysis bullosa. This shape allows at any skin damage scars behind. This causes (often severe) deformities and restrictions. The skin is by the scars shorter and stiffer. Joints stiffen. Also the mouth and tongue are sometimes less mobile by scars. Internal blisters and scars (for example in the esophagus) can cause problems, such as large a narrowed esophagus and nutrient deficiencies. Fingers and toes can bone on its own. However, there are also relatively mild forms of Dystrophic EB.
Heredity with epidermolysis bullosa (EB disease)
All forms of epidermolysis bullosa (EB) are hereditary. There are ten genes discovered that cause the disease.
For genetic counseling in your individual situation, you should consult with a clinical genetics department at a university hospital.
During pregnancy, you can have it done a CVS test to determine whether your child has the disease.
Treatment of epidermolysis bullosa (EB disease)
There is no treatment that can cure epidermolysis bullosa. The only thing you can do is take care of the skin intensively. That can be a difficult task at EB. It sometimes takes a few hours a day. In milder forms, it is usually enough to pierce the blisters and to connect the wounds properly. People with severe forms often require nursing.
There are creams, ointments and bath oils for pain, itching and infections. You can discuss with your doctor the means best suited to you.
It is very important that the connections are perfectly seated on the wounds. If a connection shifts, caused by the friction new blisters. Because of the open wounds, it may result in inflammations.
Many children with EB disease have anemia and therefore need extra iron. The shape of the power supply needs to be modified, for example blisters in the throat or esophagus. A dietitian can help.