|
| Fig. 6.4 A and B, Junctional epidermolysis bullosa, lethal type. Chronic central erosions are characteristic. |
At birth, a few blisters may be present over areas of trauma, and it may be impossible to tell this type from EB simplex or the dystrophic type. However, infants may have oral erosions that are not found in EB simplex. Junctional EB includes localized variants that are not as involved and a severe generalized form (lethal) that typically results in death prior to the age of two (Fig. 6-4). As with EB simplex, blisters and erosions over areas of trauma are common. In contrast to those with EB simplex, children with the lethal form of JEB have prominent central facial erosions with hypergranulation tissue that is difficult to treat. They also have dental defects, nail dystrophy, and tracheal and bronchiolar involvement producing respiratory distress, which is often the cause of death. The other common cause of death in infants with the lethal JEB is sepsis.
