Epidermolysis Bullosa

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by mohitaspal
Last updated 5 years ago

Discipline:
Health & Fitness
Subject:
Health
Grade:
9

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Epidermolysis Bullosa

Epidermolysis Bullosa

Blistering of the feet as a result of walking (in a pateint suffering from Epidermolysis Bullosa).

•Junctional EB

Epidermolysis Bullosa (EB), is a life-threatening skin disease typically passed through a dominant gene found in one parent which results in extremely fragile skin that blisters and tears. Blisters and areas of skin loss (erosions) occur in response to minor injury or friction, such as rubbing or scratching. For those with a severe case of this condition, even extensive walking may leave blisters and bruises.

•Dystrophic EB

•EB Simplex

Epidermolysis Bullosa acquisita (EBA)

Faulty genes are involved in the formation of thread-like fibers (hemidesmosomes) that attach your epidermis to your basement membrane. This gene defect causes tissue separation and blistering in your basement membrane zone. This disorder is autosomal reccessive.

Apparent at birth or early childhood where the faulty genes are involved in the production of a type of collagen, a strong protein in the fibers that hold the toughest layer of your skin together. As a result, the fibers are either missing or nonfunctional. Dystrophic EB can be either dominant or recessive.

Rare type of EB which is not inherited. This condition occurs as the immune system attacks healthy tissue. It is associated with Crohn's disease.

Most common mildest form of EB. Affects mainly the palms of the hands and soles of the feet. The faulty genes are those involved in the production of keratin, a fibrous protein in the top layer of skin. The condition causes the skin to split in the epidermis, which produces blisters. This disorder is autosomal dominant.

Dr. Jim Sears Visits the Boy with the "Butterfly Skin"

SOURCEShttp://www.mayoclinic.org/diseases-conditions/epidermolysis-bullosa/basics/causes/con-20032497http://ghr.nlm.nih.gov/condition/epidermolysis-bullosa-simplex

EB Pedigree reveals the autosomal dominant inheritance pattern of this disease

EB FACTSEB is estimated to affect around 1/40,000 people worldwide.There is no cure for Epidermolysis Bullosa; treatment focuses primarily on preventing infections and reducing pain.Patients are soaked with diluted vinegar solution used as a disinfectant.Scarring of the esophagus may make eating difficult and surgery may be needed to dilate the esophagus. Adults with EB are at a higher risk for skin cancer.Infant death is common as blistering of the internal organs is fatal.


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