Chronic wounds in patients with epidermolysis bullosa who ha
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Epidermolysis bullosa (EB) is a group of genetic disorders with abnormally fragile skin. Recessive dystrophic EB (RDEB) is a particular subtype in which the skin lacks type VII collagen and cannot form anchoring fibrils essential for skin integrity. Blisters turn into painful, scarring wounds that persist and recur despite standard treatments. Skin grafting using the patient’s own skin does not solve the problem, and healthy donor skin is rejected. Better treatments are needed.

Bone marrow transplantation (BMT) has been tried in RDEB with limited success. Immune-suppressing drugs, given after BMT to prevent rejection, are stopped when the patient becomes tolerant of the donor cells. Perhaps those patients might now tolerate skin grafts from their original bone marrow donor.

As part of a study of BMT in RDEB, doctors in Minnesota, USA tested this, using a CelluTomeTM device to harvest 5?cm2 skin patches from donors. Starting 6 months after BMT they grafted up to nine wounds in each of eight patients with RDEB. Donors and patients found the outpatient procedures acceptable. Treated wounds became on average 75% smaller after 6 weeks, 95% smaller after 12 weeks and completely healed at 1 year. A skin biopsy from a grafted wound at 12 months showed healthy type VII collagen and normal anchoring fibrils, with 42% of donor cells compared with 16% in nongrafted skin. This method also healed surgical wounds following an operation on RDEB scarred hands.

The authors conclude that grafting of chronic RDEB wounds with BMT donor tissue is simple, safe and successful.

Source: https://onlinelibrary.wiley.com/doi/10.1111/bjd.20104?af=R
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