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Epidermolysis Bullosa - Definition, Causes, Symptoms and Treatment

 

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Epidermolysis bullosa is the name given to a few genetic diseases causing fragile skin. Most people with epidermolysis bullosa have inherited the condition through faulty genes they receive from one or both parents. Genes are located in the body's cells and determine inherited traits passed from parent to child. They also govern every body function, such as the formation of proteins in the skin. The skin is so fragile in people with EB that even minor rubbing may cause blistering. At times, the person with epidermolysis bullosa may not be aware of rubbing or injuring the skin even though blisters develop. Depending on the type present, epidermolysis bullosa varies in severity from minor blistering of the skin to a lethal form involving other organs. The condition generally starts at birth or soon after that. Epidermolysis bullosa acquisita, however, usually appears in adults over age 50, although it has been reported in children. Severe cases of this condition involve widespread blistering that can lead to infections, dehydration and other medical problems. Severe cases may be life-threatening in infancy.

Epidermolysis bullosa usually occurs at birth or shortly after. Males and females are equally affected. Occasionally EB may be mild enough at birth not to be apparent and it is not until the child is older or reaches adulthood before it is detected. The hallmark of these conditions is the formation of large, fluid-filled blisters that develop in response to minor trauma. Some infants may have large blisters at birth. Others start shortly after birth. The classification of epidermolysis bullosa is complicated. Even within the main types mentioned, there are many different subtypes of this condition. For example, Weber-Cockayne is the most common form of epidermolysis bullosa simplex. All of the different types of epidermolysis bullosa are generally inherited. Therefore, having a family history of the disease, especially an affected parent, is a risk factor. The inheritance pattern may be dominant or recessive. The goal of treatment is to prevent the formation of blisters and subsequent complications. The intensity of care depends upon the severity of the disease. Recommendations often include measures to avoid skin trauma and to avoid high environmental temperatures.

Causes of Epidermolysis bullosa

The understanding of the causes of epidermolysis bullosa has grown greatly over recent years. All forms of EB are genetic in origin and the genes responsible for several different sub types of the condition are now known. Others still await identification. It is caused by a mutation in the genes that dictate the way our bodies produce skin. It is always inherited, but the different forms of the disorder are passed from parents to children in different ways, which are still being researched.

Common causes and risk factors of Epidermolysis bullosa:

  • Family history of Epidermolysis bullosa.
  • A mutation in the genes.
  • The disease results when skin layers separate after minor trauma.
  • Defects of several proteins within the skin are at fault.

Signs and Symptoms of Epidermolysis bullosa

Symptoms depend on the type of Epidermolysis bullosa, and which layer of the skin cells is affected. The blistering, which is the most common symptom, may cause problems ranging from minor disruption to life-threatening illness. For epidermolysis bullosa acquisita, there is an association with crohn's disease and, possibly, lupus. Symptoms of these conditions may be present, therefore, in adults who present with this type of epidermolysis bullosa.

Sign and symptoms may include the following :

  • Dental abnormalities such as tooth decay.
  • Tiny white bumps.
  • Blistering around the eyes and nose.
  • Nail loss or deformed nails.
  • Alopecia.

Treatment for Epidermolysis bullosa

Persons with mild forms of Epidermolysis bullosa may not require extensive treatment. However, they should attempt to keep blisters from forming and prevent infection when blisters occur. Recommendations might include regular whirlpool therapy and application of topical antibiotics to these wound-like areas. Your health care provider will let you know if a bandage or dressing is required, and what type. Good dental hygiene is very important, including regular dental visits. It is best to be followed by a dentist with experience treating those with epidermolysis bullosa.

Treatment may include:

  • Apply lotion to the skin to reduce friction and keep the skin moist.
  • Try to avoid using nappies in infants with severe EB, instead place child on a clean pad.
  • An oral antibiotic to reduce the growth of bacteria.
  • Epidermolysis bullosa acquisita may be treated with oral steroids and medication that suppresses the immune system.
  • Other treatments under investigation for epidermolysis bullosa include protein and gene therapy.