Epidermolysis bullosa (ep-ih-dur-MOL-uh-sis buhl-LOE-sah) is a group of rare diseases that cause fragile, blistering skin. The blisters may appear in response to minor injury, even from heat, rubbing, scratching or adhesive tape. In severe cases, the blisters may occur inside the body, such as the lining of the mouth or the stomach.
Most types of epidermolysis bullosa are inherited. The condition usually shows up in infancy or early childhood. Some people don't develop signs and symptoms until adolescence or early adulthood.
Epidermolysis bullosa has no cure, though mild forms may improve with age. Treatment focuses on caring for blisters and preventing new ones.
Source: http://www.mayoclinic.com
Epidermolysis bullosa signs and symptoms vary depending on type. They include:
Epidermolysis bullosa blisters may not appear until a toddler first begins to walk or until an older child begins new physical activities that trigger more intense friction on the feet.
Contact your doctor if you or your child develops blisters, particularly if you don't know the reason for them. For infants, severe blistering can be life-threatening.
Seek immediate medical care if you or your child:
Source: http://www.mayoclinic.com
Epidermolysis bullosa is usually inherited. The disease gene may be passed on from one parent who has the disease (autosomal dominant inheritance). Or it may be passed on from both parents (autosomal recessive inheritance) or arise as a new mutation in the affected person that can be passed on.
The skin is made up of an outer layer (epidermis) and an underlying layer (dermis). The area where the layers meet is called the basement membrane. The various types of epidermolysis bullosa are largely defined by which layer the blisters form in.
The main types of epidermolysis bullosa are:
Source: http://www.mayoclinic.com
A doctor may suspect epidermolysis bullosa from the appearance of the affected skin. He or she will likely have your child undergo laboratory tests to confirm the diagnosis. They may include:
Source: http://www.mayoclinic.com
Complications of epidermolysis bullosa may include:
Source: http://www.mayoclinic.com
It's not possible to prevent epidermolysis bullosa. But you can take steps to help prevent blisters and infection.
Source: http://www.mayoclinic.com
You can take steps at home to care for blisters and prevent new ones from forming. Learn how to care for wounds and provide good nutrition, which are essential to people with epidermolysis bullosa.
Your doctor can show you how to care for blisters properly and advise you on ways to prevent them. Talk to your doctor about safe ways to break and drain blisters before they get too large. Your doctor can also recommend products to help keep the affected areas moist, which helps promote heling and prevent infection.
In general, take these steps:
A varied, nutritious diet promotes growth and development in children and helps wounds heal. If blisters in the mouth or throat make it difficult for your child to eat, here are some suggestions:
Source: http://www.mayoclinic.com
Treating and preventing blisters and complications of epidermolysis bullosa can be stressful for you, your child and family members. You may find it helpful to share concerns and information with families in similar circumstances. Ask your health care providers about epidermolysis bullosa support groups in your area. If joining a support group isn't for you, ask about counselors, clergy or social workers who work with families coping with epidermolysis bullosa.
Source: http://www.mayoclinic.com
Having a family history of epidermolysis bullosa is the major risk factor for developing the disorder.
Source: http://www.mayoclinic.com
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