Epidermolysis Bullosa - Definition, Causes, Symptoms and Treatment
Epidermolysis bullosa (EB) encompasses a group of otherwise heterogeneous diseases mainly characterized by the presence of extremely fragile skin and recurrent formation of blister which results from minor mechanical friction or trauma. It is the prototypic mechanobullous disease.This condition is not contagious.
The disease may occur in the absence of any family history that means nobody in the family is affected in the past by the disease.This may results in the wounds over the joints on the hands, feet and other parts of the body.It generally present at the time of birth or shortly after. Exceptionly it occurs in mild cases of EBS,which may remain undetected until adulthood or usualy remain undiagnosed.
Types of Epidermolysis Bullosa:-
- Epidermolysis bullosa simplex
- Junctional epidermolysis bullosa
- Dystrophic epidermolysis bullosa
- Hemidesmosomal epidermolysis bullosa
The common symptoms are:
- Minor trauma results in blistering of the skin or temperature change.
- Blistering may be present at the time of birth.
- Blistering may be present in or around the mouth, throat,eyes and nose.Blistering results in feeding difficulty and swallowing difficulty.
- Recurrent blistering is also annoying but not life threatening.
- Epidermolysis Bullosa may cause dental abnormalities such as tooth decay mising teeth etc.
- Milia:It is the case in which tiny white bumps or pimplesoccur.
- Epidermolysis bullosa (EB) may be a result of a dominant genetic abnormalit. Change in the DNA in the genes that dictate the way our bodies produce skin leads to the disease.
- EB Simplex can occur without any family history of the disease. Instructions are provided by the faulty genes for production of keratin, a type of fibrous protein in the top layer of skin results in splitting of skin in the epidermis, producing a blister.
- Factors such as chafing, rubbing of the skin or even increase ion room temperature may cause blisters to form.
- Some infants may have large blisters at birth. Some develops shortly after birth.
- Pidermolysis Bullosa generally caused by skin's structural abnormalities.Mostly Epidermolysis bullosa results due to the molecule abnormality.
The most effective treatments for epidermolysis bullosa are given below:-
- To prevent infection, excellent skin care is required, especially if any blistered areas become crusted or denuded.
- 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.
- Treatment includes the application of appropriate non-adherent dressings to aid wound healing and prevent further damage and infections.
- There are doctors, nurses, social workers, clergy members, psychologists, dietitians, and patient and parent support groups that can assist with care and provide relevent information and emotional support.