Epidermolysis Bullosa - is a group of inherited disorders in which skin blisters develop in response to minor injury. This condition is an inherited connective tissue disease causing blisters in the skin and mucosal membranes, with an incidence of 1/50,000. Its severity ranges from mild to lethal. It is caused by a mutation in the keratin or collagen gene. People with this condition have an increased risk of cancers of the skin, and many will eventually be diagnosed with it as a complication of the chronic damage done to the skin. (www.wikipedia.com)
There are four main types of epidermolysis bullosa: * Dystrophic epidermolysis bullosa * Epidermolysis bullosa simplex * Hemidesmosomal epidermolysis …show more content…
Immunofluorescent tests or electron microscopy is usually done on skin biopsies. Growth curves will be carefully watched in an infant who has, or is believed to have epidermolysis bullosa. If there are contractures, limb range of motion will be tested.
Treatment
* Removal of any squamous cell carcinoma that …show more content…
When there is a lot of skin injury, you may need extra calories and protein to help recovery. Work closely with a nutritionist. When there are blisters or complications in the mouth or esophagus, avoid eating hard or brittle foods such as pretzels, nuts, and chips. Eating soft foods can help prevent making the sores worse * To prevent infection take very good care of the skin, especially if any blistered areas become crusted or exposed (raw). Follow your health care provider's instructions closely. You might need regular whirlpool therapy and to apply antibiotic ointments to wound-like areas. Your health care provider will let you know if you need a bandage or dressing, and if so, what type to use. * Good dental hygiene is very important, including regular dental visits. It is best to see a dentist who has experience treating people with epidermolysis bullosa. (www.answers.com)
Complications * Anemia * Death (junctional epidermolysis bullosis has a high death rate, but other, milder forms may not reduce life expectancy at all) * Esophageal stricture * Eye disorders, including blindness * Infection, including sepsis * Loss of function in the hands and feet * Muscular