EB in Depth

EB PatientEpidermolysis Bullosa (EB) is a genetic skin disorder characterized by blister formation from mechanical trauma. There are 4 main types: EB Simplex, Junctional EB, Dystophic EB, and Kindler Syndrome, with additional sub-types identified with in each type of EB. The disorder occurs in every racial and ethnic group throughout the world and affects both sexes equally. There is a spectrum of severity, and within each type, one may be either mildly or severely affected. EB ranges from being a minor inconvenience requiring modification of some daily activities to being completely disabling and, in some cases, fatal.

Friction causes blister formation. Simply swallowing food or wearing clothing with seams can result in agonizing wounds in the more severe forms of EB. In the most severe forms any epithelial tissue may form a blister secondary to minor trauma. Some examples are the oral mucosa, the external surface of the eye, the respiratory tract as well as the gastrointestinal and genitourinary tracts. In some forms of the disease disfiguring scars and disabling musculoskeletal deformities can occur.

Since EB is a genetic disease there is currently no cure for EB. The current standard of care is supportive which includes daily wound care, specialized atraumatic dressings and pain management.

It has been estimated that 1 out of every 50,000 live births are affected with some type of EB. The disorder occurs in every racial and ethnic group throughout the world and affects both sexes equally.

 

1 About EB
2 EB Simplex
3 Junctional EB
4 Dystrophic EB
5 Wound Care
6 Various Wound Care Products
7 Other Healthcare Problems
8 Bibliography

1 About EB
1.1 Acknowledgements
1.2 EB Subtypes
1.3 Additions and Changes to the EB Vocabulary
1.4 Prevalance and Incidence of EB
1.5 Testing for EB
1.6 Genetics and How EB is inherited
1.7 Prenatal Diagnosis for Epidermolysis Bullosa

2 EB Simplex
2.1 What is the Cause of EB Simplex?
2.2 How is EB Simplex Inherited?
2.3 Weber-Cockayne Subtype of EB Simplex or EBS-WC
2.4 Koebner Subtype of EB Simplex or EBS-K
2.5 Dowling Meara Subtype of EB Simplex or EBS-DM
2.6 Common Manifestations of EB Simplex
2.7 Uncommon Manifestations of EB Simplex
2.8 Rare Manifestations of EB Simplex
2.9 Other Subtypes of EB Simplex

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3 Junctional EB
3.1 What is the Cause of Junctional EB?
3.2 How is Junctional EB Inherited?
3.3 Junctional Herlitz EB or JEB-H
3.4 Junctional Non-Herlitz EB or JEB-nH
3.5 Junctional EB with Plyoric Atresia or JEB-PA
3.6 Common Manifestations of Junctional EB
3.7 Rare Manifestations of Junctional EB

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4 Dystrophic EB
4.1 What is the Cause of Dystrophic EB?
4.2 How is Dominant Dystrophic EB Inherited?
4.3 Dominant Dystrophic EB or DDEB
4.4 Transient Bullous Dermatosis of the Newborn (TBDN)
4.5 How is Recessive Dystrophic EB Inherited?
4.6 Recessive Dystrophic EB or RDEB
4.7 Recessive Dystrophic EB Inversa
4.8 Common Manifestations of Dystrophic EB
4.9 Rare Manifestations of Dystrophic EB
4.10 Skin Cancers and Dystrophic EB

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5 Wound Care
5.1 How wounds heal
5.2 Looking at the wound bed in non-healing wounds
5.3 Bathing with EB

6 Various Wound Care Products
6.1 Getting the Wound Care Products you need
6.2 Cleansers and Topical Products:
6.3 Dressing Basics
6.4 First or Contact Layer Dressings
6.5 Secondary Dressings

7 Other Healthcare Problems
7.1 Nutrition
7.2 Dysphagia
7.3 Esophageal Stricturing
7.4 Gastroesophageal Reflux
7.5 Lactose Intolerance
7.6 Constipation
7.7 Anemia
7.8 Dental Problems
7.9 Scarring, Contractures, and Syndactyly
7.10 Eye Problems
7.11 Immunizations
7.12 What Does the Future Hold for a Patient with EB?
7.13 Support Groups
7.14 What Can Research Tell Us About EB?

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8 Bibliography
8.1 Bibliography

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