Epidermolysis Bullosa

Epidermolysis bullosa is a group of inherited disorders in which massive blistering of the skin develops in response to minor trauma. This blistering of the skin can come about as the result of the gentlest friction, and many common activities, such as rubbing or knocking the skin, can lead to large painful blisters. At times, the person with EB may not be aware of rubbing or injuring the skin even though blisters develop. They may develop inside the body, in such places as the linings of the mouth, esophagus, stomach, intestines, upper airway, bladder, and the genitals. The epidermis is the outermost layer of skin and "lysis" refers to a breakdown of the structure within this layer. People with EB have extremely delicate skin, and children with the illness have been described as "Butterfly Children", as their skin is considered to be as fragile as a butterfly's wing. EB can take over 20 different forms, each of which has characteristic symptoms. Some forms of EB are quite mild, but others can be more severe and incapacitating.

Epidermolysis bullosa (EB) is the name given to a few genetic diseases causing fragile skin. There are thought to be approximately 5,000 people in the UK who have one or other form of EB. The inherited form of EB has three major subtypes-EB simplex (EBS), junctional EB (JEB), and dystrophic EB (DEB)-based on whether blisters arise in the epidermis or in the middle (i.e., the lamina lucida) or undersurface of the lowermost portion (i.e., the lamina densa) of the dermoepidermal junction, respectively. It is present from birth although in some cases it may not show up until a little later in life. It affects both sexes, and is not specific to any racial group. There is an extremely rare non- genetic form of EB known as EB Acquisita, however most clinicians see this as a separate and distinct disorder.

Causes of Epidermolysis Bullosa

The common Causes of Epidermolysis Bullosa :

Factors such as chafing, rubbing of the skin or even increase ion room temperature may cause blisters to form.
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.
Pidermolysis Bullosa generally caused by skin's structural abnormalities.Mostly Epidermolysis bullosa results due to the molecule abnormality.
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.

Symptoms of Epidermolysis Bullosa

Some Symptoms of Epidermolysis Bullosa :

Nail loss or deformed nails.
A family history of epidermolysis bullosa or an affected parent.
Blistering present at birth.
Alopecia (hair loss).
A hoarse cry, cough, or other respiratory difficulties.
Blistering around the eyes and nose.
Blistering in or around the mouth and throat with feeding difficulty or swallowing difficulty.

Treatment of Epidermolysis Bullosa

Treatment includes the application of appropriate non-adherent dressings to aid wound healing and prevent further damage and infections.
Proper nutrition is also important. When skin injury is extensive, increased calorie and protein intake may be necessary to help recovery.
Good dental hygiene is very important, including regular dental visits.
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.

Nails Care

Anychia
Clawing of the Nails
Clubbing
Discoloration of the Nail Plate
Ingrowing Toe Nail
Koilonychia
Longitudinal Striations
Loss of Nail Plate
Onycholysis
Paronychia
Pitting of Nail Plates
Pterygium
Thickening of Nail Plate
Transverse Ridges

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