Rare Diseases Symptoms Automatic Extraction

Evaluation of wound care options in patients with recessive dystrophic epidermolysis bullosa: a costly necessity.

[dystrophic epidermolysis bullosa]

Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic disorder in which mutations in collagen VII, the main component of the anchoring fibril, lead to skin fragility and to the development of acute and chronic wounds. Wound care and dressing changes are an important part of the daily lives of individuals with RDEB. Ideal wound care should improve wound healing, minimize pain, and improve quality of life. The objective of the current study was to review wound care options that might be used in a patient with RDEB and calculate the cost of these various options based on publicly available pricing of wound care products. There is a wide range of costs for wound care options in patients with RDEB. For example, a 1-day supply of dressing for a neonate boy with RDEB ranges from $10.64 for the least expensive option to $127.54 for the most expensive option. Wound care in patients with severe, generalized RDEB has not only a significant economic effect, but also directly affects quality of life in this patient population. Although randomized controlled trials evaluating different wound care products in patients with RDEB are lacking, small studies and expert opinion support the use of specialized nonadherent dressings that minimize skin trauma and promote wound healing. Until there is a cure, prospective studies are needed to assess pain, quality of life, and wound healing associated with the use of specialized wound care products for this life-altering condition.