What is Epidermolysis Bullosa Simplex?

Epidermolysis bullosa simplex is a genetic disorder that causes frequent outbreaks of blisters and irritation on the skin that are usually confined to the hands and feet. People who have the condition typically experience recurring emergences of red, white, or dark-colored blisters that break open and heal within a few days. Epidermolysis bullosa simplex is inherited and symptoms often start to appear shortly after birth. Treatment depends on the severity of symptoms, but most parents and patients can learn how to care for blisters at home and prevent complications.

There are several different forms of epidermolysis bullosa, and the simplex variety is generally the mildest. Genetic mutations affect the production of keratin in the outermost layer of skin. As a result, the epidermis is especially fragile and only loosely held together. Epidermolysis bullosa simplex is an autosomal dominant disorder, which means that only one parent needs to possess the mutation in order to pass it down to offspring.

Pus-filled blisters can erupt on the skin of a person with epidermolysis bullosa simplex spontaneously. Friction from clothes or bedding, excessive heat or cold, and sweating are possible triggers that can make the condition worse. Blisters do not normally cause a great deal of pain but they can feel tender and make certain activities uncomfortable. Epidermolysis bullosa simplex is most often isolated on the palms of the hands or the soles of the feet. It is possible, however, to experience widespread outbreaks on the legs, arms, torso, scalp, or face.

An infant who develops blisters should be evaluated by a pediatrician to check for signs of epidermolysis bullosa simplex and other types of skin disorders. In addition to performing a physical evaluation, a doctor usually collects a small sample of skin and blood so that genetic tests can be performed. There is no clear cure for epidermolysis bullosa simplex, though a physician can provide information about symptom management techniques once the diagnosis has been confirmed.

Parents of children diagnosed with epidermolysis bullosa simplex are instructed to carefully monitor changes in the appearance or frequency of blisters. Having their children wear loose-fitting shoes, socks, and clothing may be able to prevent rapidly recurring outbreaks. Blisters tend to heal faster when they are drained, so parents may be given instructions on how to safely pop them with sterile needles and dress wounds to prevent infection. Older children, adolescents, and adults with the condition are given helpful tips on proper hygiene and skin care.