EBS Weber-Cockayne

EBS Weber-Cockayne (EBS-WC) also is known as localized EBS. Blisters occur primarily on the palms and soles, although any part of the skin may blister in response to friction or trauma. It is not unusual to develop blisters on other parts of the body when there is increased friction on the skin, such as with horseback riding, which may cause blisters on the buttocks and thighs. Another common problem are blisters due to tight clothing or waistbands. Blisters occur more frequently when the weather is hot and humid. During summer, when the pavement is hot, many people report developing blisters on their feet after walking from a parking lot into a store or work.

EBS-WC usually presents during infancy or early childhood, when the baby is kicking in the crib or starting to crawl or walk. Sometimes, it does not appear until the individual is older; it even has been reported as appearing in adults who, after joining the military and being required to march long distances, developed blisters.

Thickening of the skin on the palms and soles (keratoderma) may occur in some adults with EBS-WC and, occasionally, fingernails and toenails become thickened and dystrophic (abnormal in appearance) with recurrent blistering. Blistering usually is confined to the skin, except during infancy, when oral blisters may develop due to sucking during feeding or when a pacifier is used. Milia and scarring do not usually occur, and there is no increased incidence of dental caries (cavities) or dental enamel problems. Affected children typically do not have poor growth, anemia, or problems with the trachea/respiratory tract, intestines/gastrointestinal tract, eyes or genitourinary tract. There is no increased risk of skin cancer.