Epidermolysis bullosa simplex

Epidermolysis bullosa simplex, often shortened to EBS, is an inherited skin disorder in which the skin is unusually fragile and blisters easily, even after very light friction or rubbing. It is one of the major forms of the wider epidermolysis bullosa group of genetic conditions. EBS results from mutations in the genes that code for keratin 5 and keratin 14, two structural proteins of the outer skin, and these changes stop the epidermis from bonding properly to the dermis beneath it at the dermal-epidermal junction. A small number of patients carry no mutation in either keratin gene, and changes in the plectin gene are being studied in the Ogna form. The condition is divided into several types. There is no cure, and care aims to protect the skin, prevent blistering, promote healing and avoid complications, including keeping the patient cool and reducing friction.