A VILLAGE WHERE SUN IS A TABOO.

Araras in the Brazillian state of Sao Paolo resembles a ghost town because most residents suffer from Xeroderma Pigmentosum (XP), which means their skin is unable to repair damage caused by ultraviolet light.

A villager, Djalma Jardin, had just one eye which he was unable to close and he spent his days indoors due to a rare genetic disorder.
Of the 800 people in the village, 600 are affected by XP. Of those, 20 have full-blown symptoms and are susceptible to an aggressive form of skin cancer.
Jardin’s face was ravaged by the disease, leaving him with just one eye. He slept with a plaster over it, because the damage to his eyelid meant he could not close it.

“If I go out I feel the sun burning me,” he said. ‘”I go to bed and wake up the next day with a small spot, and then in a couple of days it’s growing quickly, like the one I got in my eye which never stopped growing. It’s an awful disease, terrible.”
Following the interview, Jardin’s condition worsened and the illness sadly cost him his life. His family has already lost another relative to the disorder and another of Jardin’s brothers and his sister Claudia are also affected by it.

Deide, a dairy farmer, has also lost much of his face to the incurable condition. He said: “I had surgery to remove the palate and the right jawbone. Without the prosthesis I couldn’t talk.”

Gleice Machado, a store owner, was keen to find out what was causing the epidemic, after seeing so many of those affected coming through her doors.
She said: “I had no idea what it was and why they were disfigured,” adding: “Some believed it was a sexually transmitted disease, others said they believed this was a punishment from God.”

Gleice raised the awareness of the condition through the local press, and the story was picked up by the national media.

Dr Carlos Menck, a Sao Paulo-based genetics biologist, recognised the condition and was keen to know why it was so rampant in the village. He said:“We went to the area and tried to identify genetic mutations affecting the patients.“Until not long ago, people believed it was a contagious disease, but it’s an inherited disease.”
Dr Menck and his team discovered 600 of the 800 villagers were carrying a recessive XP gene, after running tests on all of the villagers.

The biologist said: “It is not possible to cure XP immediately, till perhaps in the future, maybe in 20 or 30 years time.” The dermatologists infer that the disease could be traced back to the families of three Portuguese settlers.
In Araras there is a concentration of people with the faulty gene who keep marrying each other, so the gene becomes dominant and never seem to dis appear.

There is no cure for XP, but doctors have now warned villagers to stay out of the sun completely. Hopefully, this advice will save many lives.