Title

Inpatient management of children with recessive dystrophic epidermolysis bullosa: A review

UMMS Affiliation

Department of Pediatrics, Division of Critical Care; Department of Dermatology

Date

9-25-2017

Document Type

Article

Disciplines

Dermatology | Pediatrics | Skin and Connective Tissue Diseases

Abstract

Recessive dystrophic epidermolysis bullosa is a disorder marked by skin and mucosal blistering after minimal trauma. Even the most routine procedures in the hospital, if done incorrectly, can precipitate extensive skin loss, pain, and scarring. Most providers have little experience working with patients with this degree of skin fragility. When a person with recessive dystrophic epidermolysis bullosa is admitted to the hospital, there are multiple considerations to keep in mind while strategizing an effective care plan: avoidance of new blisters with a "hands-off" approach; careful consideration of all indwelling devices; symptomatic management of pain, itch, and anxiety; coordination of dressing changes; aggressive treatment of skin infections; environmental and staffing considerations; and awareness of other chronic complications that affect care, such as anemia, malnutrition, and chronic pain. To minimize discomfort for patients with recessive dystrophic epidermolysis bullosa during the hospital stay, inpatient care teams should understand these considerations and modify the care plan accordingly. Prior preparation by the hospital facility and inpatient care team will facilitate the delivery of safe and effective care and greatly improve the overall patient experience.

Rights and Permissions

Citation: Pediatr Dermatol. 2017 Sep 25. doi: 10.1111/pde.13276. Link to article on publisher's site

Related Resources

Link to Article in PubMed

Keywords

epidermolysis bullosa, health care delivery, immunobullous disease, quality of life

PubMed ID

28944966