Introduction

Ehlers-Danlos syndrome (EDS) is a group of heritable disorders with an incidence of approximately 1 in 5000 births.[1] It is associated with a defect in collagen formation leading to skin fragility and hyperelasticity of skin, hypermobility of joints, and poor wound healing with scarring.[2,3] It has been subdivided into separate types according to the predominant areas affected and the degree of abnormality (Table 1). Collagen plays an important part in the wound healing process, and patients with EDS have suboptimal wound healing and multiple complications following surgery secondary to defects in collagen metabolism.[4–6]

The authors treated two patients with traumatic wounds of the lower extremities with a bioengineered skin equivalent (BSE) (Apligraf®, Organogenesis, Canton, Massachusetts). Both of these patients failed to heal their wounds normally with standard wound care. BSE consists of bovine collagen and human fibroblasts and keratinocytes.[7] The fibroblasts and keratinocytes in Bse produce growth factors and antibiotic peptides creating a physiological microenvironment that can stimulate wound healing.[8]

Case Reports

Case report #1. Patient #1 was an 11-year-old male with a history of EDS type II diagnosed at 10 years of age. His skin was flexible and loose around the joints. He was prone to large subcutaneous hematomas, and his skin was fragile and would easily tear after minor trauma. The skin held sutures, and his wounds healed with scarring. He presented to our office with a one-week-old soft tissue injury over the anterior aspect of his left lower extremity. This occurred while playing soccer. There was significant skin loss over the area with resultant eschar formation. On initial examination, the eschar had separated from the 5cm x 5cm wound bed, which contained some underlying granulation tissue (Figure 1A). The initial management consisted of wet-to-dry dressings with normal saline solution.

One week later, the patient returned to the office. The wound bed appeared healthy with continued presence of granulation tissue but without other signs of healing or wound contraction. Due to suboptimal healing, the patient was taken to the operating room one week later where BSE was placed onto the wound (Figure 1B). A sterile dressing was applied, and a splint was fabricated for immobilization.

The BSE was examined on post-operative Day 5 and appeared viable with adherence to the underlying wound. Daily dressing changes consisted of application of antibiotic ointment, nonstick gauze, and the splint. Weekly follow-up visits continued, and by post-op Day 30, the wound was completely healed with good cosmetic results (Figure 1C).

Case report #2. Patient #2 was a nine-year-old female with EDS type I diagnosed at two years of age. She had the typical features of velvety skin that is hyperelastic and fragile. Her skin would tear, even after minor trauma, and would not hold sutures. Her parents reported that these minor wounds might take up to four months to heal and there was unsightly scarring of the healed wounds. She had a history of bilateral hip dysplasia and a left club foot. This patient presented to the authors’ office four weeks after a sutured repair of a laceration of the posterior aspect of the right lower extremity. The wound had dehisced. Necrotic tissue at the inferior aspect of the wound was debrided after suture removal, and daily dressings of collagenase and polysporin powder were prescribed and continued at home. On a follow-up visit a week later, the wound measured 3cm x 5cm with a granulating base (Figure 2A).

Two weeks after the first visit and after re-evaluation and determination of poor healing, the wound was debrided in the operating room and Bse was placed onto the wound (Figure 2B). Antibiotic ointment and a nonstick gauze dressing were applied.