Online Medical Clinic

Epidermolysis Bullosa Simplex Treatment

October 11, 2007 on 11:24 am | In Skin DIsorders |

Epidermolysis Bullosa (EB) is a unusual genetic disease differentiated by extremely fragile skin. EBS with mottled pigmentation; EBS, Dowling-Meara type), which can be severe. The signs and symptoms of this condition vary widely among affected individuals. Blistering may mainly affect the hands and feet in mild cases, and blisters usually heal without leaving scars.

Other marks may involve thickened skin on the palms of the hands and soles of the feet; rough, thickened, or absent fingernails or toenails; and blistering of the soft tissues inside the mouth. EBS is almost exclusively a regnantly inherited condition seen equally in males and females. This means anyone who has EBS can transmit the condition onto his or her children.

This condition is approximate to affect 1 in 30,000 to 50,000 people. EB is caused by a missense mutation in either of the KRT5 or KRT14 genes. Genetic counseling is present for couples affected with EBS. The mutation locates on the keratin filament with harshness of EB. Weber-Cockayne is the dullest form with delayed onset and blisters usually isolated to the hands and feet.

Koebner is more harsh with generalized blistering with onset at birth or sometimes later. Antiseptic baths, saline compresses and topical antibiotics are usually used in order to offer relief and prevent secondary infections in areas of blistering. Topical corticosteroids are also used in principle, their mechanism of action lowers down reepithelization.

Tense blisters can also be evacuated for symptomatic relief. In patients with EBS, the mutations that code for the amino terminus of keratin 5 are associated with mottled pigmentation. Protein replacement environs the production of a defective protein by recombinant techniques and topical application in a lotion, or cream, form. Gene therapy would offer cultured keratinocytes, or stem cells, directly into patients with a specific gene defect.

Nutritional assessment taking these factors into consideration is essential for replenishing the malnourished patient. Oropharyngeal and GI lesions greatly risks the nutritional well being of patients having EB. Avoiding activities which traumatize the skin (e.g., hiking, mountain biking, contact sports) can lessen skin damage.

Epidermolysis Bullosa Simplex - Prevention and Treatment Tips

1. Antiseptic baths, saline compresses and topical antibiotics are commonly used in order to provide relief.

2. Topical corticosteroids are also used; in principle, their mechanism of action slows down reepithelization.

3. Tense blisters can also be evacuated for symptomatic relief.

4. Genetic counseling is present for couples affected with EBS.

5. Avoiding activities which traumatize the skin

6. Nutritional assessment taking these factors into account is essential for replenishing the malnourished patient.

7. Oropharyngeal and GI lesions greatly threaten the nutritional well being of patients with EB.

8. Topical application in a lotion, or cream is also medicate epidermolysis bullosa simplex.