80% of His Skin Had Been Lost. Doctors Grew Him a Replacement

80% of His Skin Had Been Lost. Doctors Grew Him a Replacement

The German doctors realized they had to do something drastic or their seven-year-old patient would die. (About 500,000 people worldwide have the condition.) The boy in today's study developed a bacterial infection that made him lose skin on over two-thirds of his body, leaving him bright red and raw.

A team of biologists who specialize in gene therapy came in from University of Modena and Reggio Emilia in Italy, and the boy's parents agreed to an experimental therapy. "To replace and gene-correct the whole skin of a patient is just unbelievable". But after almost a decade of filing paperwork and improving his skin cell production system, De Luca was ready to try. Although the body can take care of most cuts or blemishes fairly well, once large portions of the skin become compromised through burns or diseases like JEB, it's unable to fulfill these duties. The scientists then genetically modified these skin cells, using a virus to deliver a healthy version of the LAMB3 gene into the nuclei. The engineered skin cells and stem cells were then used to grow skin in the lab. This matches up with the experience of burn victims who receive skin grafts, according to de Luca.

Plastic surgeon Professor Tobias Hirsch, from Bochum Children's Hospital, described the critical condition of the boy when he was admitted to the burns unit.

The treatment, outlined in a paper in the journal Nature, involved first taking a sample from the patient's remaining healthy skin. "After this 10 years of struggling to accomplish all these rules and paperwork and bureaucracy, the moment you see a patient like this, you understand it was worth it to do it".

The genetically modified cells in the graft include specialised skin stem cells that meant once the transplant was integrated it was able to renew and sustain the healthy skin.

Writing in Nature, the scientists said: "Transgenic epidermal stem cells can regenerate a fully functional epidermis virtually indistinguishable from a normal epidermis".

Other researchers in the field say they are impressed with the team's results.

"What is nice about this study is the combination of gene and cell therapy together", said Higgins, whose own work focuses on skin regeneration. Additionally, because the team modified stem cells-which are essentially the starter-cells that create every type of other cells-his new skin grows and replaces itself just like it does in healthy patients.

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Some unusual pigmentation remains on the boy's skin because of the earlier damage. De Luca said the boy will be monitored closely for skin cancer and other potential issues. "When we engrafted him he was 17 kilograms [37 pounds]", he adds.

Prof Hirsch added: "If he gets any bruises they just heal as normal skin heals".

Epidermolysis bullosa prevents the outermost layer of the skin, called the epidermis, from anchoring to the underlying skin layer (the dermis) correctly. The boy has no scarring, because his disease was literally only skin-deep.

Needless to say, epidermolysis bullosa significantly reduces a patient's quality of life. This is why De Luca had to correct the gene before growing new skin for the boy. The culprit is a deficiency of the protein laminin-33, and it's caused by a mutation in one of three genes.

"This takes us a huge step forward", said Dr. Peter Marinkovich of Stanford University School of Medicine, who has done related work. In his own research Marinkovich has used gene therapy to treat four patients with a different genetic form of epidermolysis bullosa.

In the future, if the treatment is shown to be safe in the long term, scientists believe the approach could be used to treat less severe skin disorders.

The boy was in the hospital for more than eight months, but now he is healthy. The complicated treatment will not be cheap. "There are very few diseases that have benefitted so far", he said. All children can run around and play, why am I not allowed to play soccer? "The sooner we do it", he explains, "the better it is for the kids".

Two years on the boy is doing well, his doctors said.

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