Epidermolysis Bullosa Simplex


Epidermolysis Bullosa Simplex (EBS) is a group of diseases with 70% of all cases being EBS.

EBS is characterised by the development of blisters following little or insignificant trauma to the skin. This occurs as there is a lack of adhesion of the skin above the basal layer which results in these blisters.

The vast majority of cases are inherited as dominant traits i.e. the parent visibly has the same traits. The generalised type of EBS occurs in 1 in 500,000 at birth. It is characterised by the development of blistering over the joints of the hands, elbows, knees and feet and other sites subject to repeated trauma. Another form of EB Simplex can present with blistering on the whole body.

Blistering can manifest itself later on in life but children can be affected at birth or shortly thereafter. They show improvement within the first few months, but the disease recurs when the child begins crawling. The blistering can also worsen during the summer and improves during the winter.

At present there are no specific therapies for any form of inherited EBS and treatment is focused on the management of the disease. So treatment is based on a prevention of both mechanical trauma (via judicious use of padded bandages and loose fitting garments) and keeping infections at bay with the use of mild topical antibiotic ointment or creams.

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The prevalence of skin disease exceeds that of obesity, hypertension, or cancer. Despite skin being the largest organ of the human body, dermatological research remains one of the most under funded areas of medicine. In a world where society has an increasing preoccupation with image and it’s importance to every aspect of a person’s life, sufferers of skin diseases are feeling and being more marginalised and isolated than ever.

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