Epidermolysis bullosa (EB) is a group of inherited bullous disorders characterized by blister formation in response to mechanical trauma. Epidermolysis bullosa simplex (EBS) is one of the major forms of epidermolysis bullosa. The signs and symptoms of this condition vary widely among affected individuals. Blistering may primarily affect the hands and feet in mild cases, and blisters usually heal without leaving scars. There are three main forms of inherited EB. These different subtypes are defined by the depth of blister location within the skin layers, and the location of the dissolution of the skin. EB Simplex (EBS) is also subtypes of epidermolysis bullosa. Blister formation of EB Simplex is within the basal keratinocyte of the epidermis. Sometimes EB simplex is called epidermolytic.



JEB is a collection of diseases characterized by intralamina lucida blistering. Mucous membranes at the level of the lamina lucida within the basement membrane zone. Dystrophic EB (DEB) forms which can lead to scarring occur in a deeper tissue level; the sub-lamina densa region (the beneath the lamina densa) within the upper dermis. Epidermolysis bullosa acquisita, however, usually appears in adults over age 50, although it has been reported in children. Also, mild cases of epidermolysis bullosa simplex may remain undiagnosed until adulthood. An estimated 50 in 1 million live births are diagnosed with EB, and 9 in 1 million are in population. EB affects males and females of all races around the world. The treatment is tailored to the severity and extent of skin/organ involvement.

The most important treatment for EB is daily wound care. Long term use of steroids for epidermolysis bullosa is generally not recommended. To prevent infection, excellent skin care is required. Good dental hygiene is very important, including regular dental visits. Proper nutrition is also important. When skin injury is extensive, increased calorie and protein intake may be necessary to help recovery. Skin grafting for denuded or ulcerated areas of the skin may be necessary. Other surgical procedures for complications of epidermolysis bullosa might be recommended as well. Epidermolysis bullosa acquisita may be treated with oral steroids and medication that suppresses the immune system. To prevent skin trauma and blistering, it may help to wear padding around trauma-prone areas .

Epidermolysis Bullosa Treatment and Prevention Tips

1. Proper nutrition is also important.

2. Genetic counseling is recommended for prospective parents.

3. Good dental hygiene is very important, including regular dental visits.

4. A topical antibiotic, such as bacitracin, mupirocin, or sulfadiazine, should be routinely applied

5. Use foam padding or sheepskins to help reduce friction on furniture such as beds, chairs and infant car seats.

6. Try to avoid using nappies in infants with severe EB, instead place child on a clean pad.