Junctional Epidermolysis Bullosa (JEB)

JEB effect on skin

In this type, blistering occurs in a skin layer called the lamina lucida within the basement membrane zone, which is situated at the junction between the epidermis (upper layer of the skin) and the dermis (lower layer). JEB is the most severe type of EB and accounts for about 5 percent of cases.

Like EBS, JEB is also inherited in an autosomal recessive manner. However, the condition develops only when the defective gene is inherited from both parents who are EB carriers but do not show symptoms themselves.

JEB exists in three forms: Herlitz JEB, a very severe form of EB that can cause serious blistering internally and is often deadly within early years of life; non-Herlitz JEB, where blistering may be mild or severe, but can cause life-long pain and be disabling; and JEB with associated pyloric atresia, a severe form that affects both the skin and digestive tract

Stages of a Newborn with JEB

Between Day 1 to Day 7 after birth

Day 7 to Day 13

Day 20 and above

This baby is suffering from JEB, and unfortunately, his life expectancy is low. The blisterings happens more often than they heal, and even internally, the baby is suffering from blisters.