Management of Epidermolysis Bullosa in the Adult Patient

Epidermolysis bullosa (EB) comprises a group of inherited diseases characterized by fragile epithelial tissues resulting in the formation of blisters or erosions following minimal trauma.  Prevalence of EB is 8.2 per 1,000,000 live births, (Laimer, Prodinger, & Bauer, 2015).  In addition, infants and children with inherited EB, particularly those with Junctional Epidermolysis Bullosa (JEB), are at significant risk of death as a result of disease complications. (Fine, Johnson, Weiner, & Suchindran, 2008) The rarity of the disease, notably in an adult, poses multiple challenges for treatment providers as much more research is needed regarding EB.

This case study examines a 45-year-old female diagnosed with JEB at 6 months old, admitted to a long term acute care hospital in July 2017. Patient presented with extensive epidermal erosions over majority of extremities and lower trunk (pictures included).

The patient’s dressing changes take two to three hours daily with multiple caregivers.  Over several months, trial and error as well as collaboration with multiple interdisciplinary clinicians have resulted in finding the optimal dressing procedure application for both the patient and the staff. Wound healing has been promoted, furthermore the amount of time taken for dressing changes to be completed, and discomfort for patient, have significantly decreased.

This case study focuses on how primary caregivers are managing patient’s manifestions of EB, most specifically wound care.  In addition, how staff had worked to enable patient to achieve discharge home.