Using a Dermal Skin Substitute in the Treatment of Chronic Wounds Secondary to Recessive Dystrophic Epidermolysis Bullosa: A Cas

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Results

Data from six patients (ages 8 years to 23 years, four girls, two boys) with chronic wounds secondary to generalized RDEB who attended the outpatient clinic and were treated were analyzed. Patient participation was determined according to need; these individuals constantly require wound care. In all cases, the diagnosis of generalized RDEB had been made shortly after birth and was confirmed by electron microscopy of skin biopsy. A skin biopsy from some of the areas being treated showed less than 1.0 x 106 colony forming units per gram of bacteria using semi-quantitative culture techniques. The skin substitute was applied as described. Only one adverse event was recorded and was deemed not to be a result of the therapy provided. At weeks 1 to 2, epidermal coverage ranged between 80% and 100%. The mean epidermal coverage at 8 weeks for all patients at all sites was 74%. Some of the sites had persistent coverage and others had subsequent breakdown.

Case Studies

Patients with EB generally have recurrent wound episodes throughout their lives — a continual cycle of breakdown and healing, regardless of precautionary measures. The patients studied here had wounds that had been present for prolonged periods of time or reoccurred frequently. Each case study focused solely on location of the wounds and the extensive range of previous treatments (see Table 2 and Table 3).

Case 1. Miss A, an 8-year-old Caucasian girl with RDEB, had wounded areas that included her face, hands, arms, torso, chest, back, and feet. Her wounds previously were treated with silver sulphadiazine cream, polymyxin-gramicidin cream, fusidic acid cream, petrolatum ointment, nanocrystalline silver antibacterial dressings, moisture balance dressings, non-adherent dressings, and gauze. No debridement was performed before skin substitute application, which she received two times. On August 28, 2001, four pieces were applied to her right anterior shin, right heel, and the dorsum of her right foot, persistently open areas before treatment. On September 11, 2001 (13 days later,) all areas exhibited signs of healing except the dorsum of the right foot remained unchanged. On September 26, 2001 (4 weeks after application) another piece of skin substitute was applied — half on the right medial malleolus and the other on the lateral side (see Figure 4a, b).

Miss A had Staphylococcus aureus cultured from her right shin on the date of application, which explained the lack of improvement when compared to other treatment sites. The wound was treated and infection resolved by the time of the next assessment. Infection was a recurring event with Miss A but did not appear to hinder skin substitute take in other areas.

A second round of application to other wounded sites was scheduled for October 24, 2001 where a piece of skin substitute was applied to Miss A’s right elbow. At the first follow-up on November 6, 2001, the wound was healing. At the next follow-up visit on December 5, 2001, the skin substitute on both the right elbow and the right knee were intact and healing.

Case 2. Mr. B, a 12-year-old Caucasian boy with RDEB, exhibited esophageal dilatation and had most of his teeth pulled because of EB in his mouth. He also had an equinus deformity of the right foot. Other wounded areas included his face, hands, arms, torso, chest, back, and feet. His wounds were previously treated with silver sulphadiazine cream, nanocrystalline silver antibacterial dressing, silicone-coated sheets, silicone non-adherent mesh, and silicone non-adherent foam. No debridement was performed before skin substitute application.

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