Neonatal junctional epidermolysis bullosa: treatment conundrums and ethical decision making

Junctional epidermolysis bullosa (JEB), generalized severe (previously called JEB, Herlitz-type) has an extremely poor prognosis, with a mean age of death at 5 months old and most dead before age 3 years. We describe a typical case of a neonate with JEB who developed failure to thrive before his death from fungal septicemia at 4 months of age. This case highlights the ethical considerations of invasive treatments such as gastrostomy tube placements, intubations, and central line placements in neonates with JEB. We review the literature as well as discuss the ethical conundrums in the care of patients with JEB and other severe forms of epidermolysis bullosa.