Health Care

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

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

A seven year old boy with a rare genetic disease called junctional epidermolysis bullosa received the most extensive skin transplant yet to treat his condition.

A boy who almost died when disease stripped most of the skin from his body, is playing football two years after he received new, gene-edited skin in an experimental procedure, the doctors who treated him said Wednesday. Suffering a double bacterial infection, the boy had been admitted to the Children's Hospital of Ruhr-University in Bochum where the infections ultimately led to the loss of approximately 80 percent of his epidermis.

"When he woke up", Dr. De Luca told the New York Times, "he realized he had a new skin". After trying everything they knew, they concluded the child would die.

"He was in severe pain and asking a lot of questions", the boy's father said in a video provided by the hospital "Why do I suffer from this disease?"

His doctors consulted experts around the world. Surgeons in Germany sent a skin biopsy to Modena, and two major skin transplants followed. The details of the case were published in the latest issue of the journal Nature.

As the discussion turned to making the boy's last days as comfortable as possible, his parents urged doctors to try "anything". Dr. Michele De Luca, director of Center for Regenerative Medicine Stefano Ferrari at the University of Modena in Italy, was approached by the team caring for the boy. The cells, which grow as sheets, were then further expanded until enough sheets were produced to cover the boy's limbs and torso.

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After permissions were obtained for the compassionate use of an experimental therapy, the skin was grafted onto the boy's body in two operations in October and November 2015.

The boy was in the hospital for more than eight months, but now he is healthy.

Nearly two years on, he is doing well and playing football, having gone from being on constant morphine to no drugs at all. Each month, human skin is entirely replaced with new cells, but whether this renewal is the result of a large population of equally potent progenitor cells or of a smaller number of individual stem cells that dominate regeneration has been hotly debated. They had previously used gene therapy to produce a small piece of skin in a similar case. He, like other people with the disease, carried a mutation in a gene that controls the integrity of the skin. JEB is incurable and only around 40 percent of the sufferers reach adolescence according to studies. Those who survive deal with chronic wounds, and often get skin cancer.

Put simply, "it's a lovely piece of work", stem cell and regenerative medicine researcher Fiona Watt of King's College London writes to The Scientist about the team's accomplishment.

The researchers grew the replacement skin from the boy's healthy epidermal cells, tweaking their genetic makeup in the lab to ensure the disease-causing mutation wasn't present.

"I'd say that this epidermis will stay basically forever", De Luca said. This project, for example, relied on long-term follow-up of a patient treated in 2006, as well as parallel studies that underpinned the development of tools for ex vivo gene therapy and for growing transplantable sheets of epidermis in vitro.