Epidermolysis bullosa is a connective tissue disease that manifests as extreme skin blisters. It is an inherited genetic disorder and has 3 main distinctive types. In case of having EB simplex there is no chance of developing dystrophic or junctional EB.
EB cause
EB is caused by genes that lack information about certain protein production in the skin top layer. This causes the proteins to deform and not support the top skin layer as they should. Because of this the upper layer of skin does not bind to the lower skin layer properly and blisters start to develop in between the two layers.
EBS is inherited indiscriminately of gender, and there is 50% chance for a child to inherit EBS if one of the parents was affected. EBS can also appear as mutation without previous family history.
There is no known cure for EBS but skin blistering can be prevented through a course of careful and patient skin care.EBS has three subtypes. These are Weber Cockayne, Kobner and Dowling Meara.
Weber Cockraine is the most common EBS type. It manifests in blisters that are localised to feet and hands. The first signs of blistering appear when the child starts to walk.
Kobner type can be obvious from birth or develop in first few weeks. Blistering appears in areas exposed to friction. Although most blisters are caused by clothing, they can also appear inside the mouth.
Blistering management
When a new blister appears, the fluid inside it should be released with sterile needle or scissors. This will prevent the further blister spread. Thrusting the blisters may be painful, in which case a finer needle with a syringe can be used.
The dressing issue can be addressed in few ways. Some people cover the blistered area with corn flour that dries the blisters and reduces friction. Another solution is clothing that does not stick. These Dressings are constantly evolving and some models are made with the cooling effect.
Dealing with pain
Blisters are known to be very painful and to influence mobility. Painkillers like ibuprofen and paracetamol can be used when the pain starts to get unbearable. Blisters can be especially bothersome during the summer and may require a steady therapy of pain management.
In some cases blisters can develop in anal region and prevent the stools coming out by pain. Children may be afraid to poo because of the pain. This state can be resolve by a diet with plenty of fluid. In occasional cases medicine can be used to soften and stimulate the stools movement through the bowel.
Although there is no sure way of stopping the blisters from forming, some measures can help to make the appearance minimal. Cooling the affected area often reduces the rate of blistering. One of the ways to do so is soak the affected areas with cool water. Temperature can be regulated indoors. Wearing natural materials and choosing proper footwear is important. Walking problem differs from day to day. Some people preserve themselves for enjoyable activities.
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