London - Few people would expect that going “white with shock” would lead to a permanent condition. But when Emma Rush sat for weeks on end at the bedside of her critically-ill newborn daughter Genevieve, the colour began to drain from her face, hands and arms — and it never came back.
Although her husband and family noticed, nothing was said, and it was only months later when her baby was out of danger that Emma realised what had happened to her.
Emma had developed vitiligo, an incurable long-term disease that causes a disfiguring loss of skin colour.
The colour completely disappeared from her hands, while her eyes and mouth were encircled by three bizarre white bands of depigmented skin leaving her looking, she says, “like a panda”.
“It gradually got worse and worse,” says Emma, 48, a successful lawyer and legal trainer who lives with her husband and two daughters in West London.
“I started off by covering it up with normal make-up, but by the second year of having it, it had spread into two large patches around each eye and another one which started under my nose and spread around my mouth.
“It has gradually moved outwards, so my face is now 60 percent affected, and there are just triangular patches of normal skin on my cheeks.
“It got to the point where I had to move to using heavy-duty prescription camouflage make-up on my face, which you have to paste on and set with powder.”
An estimated 45 million people worldwide suffer vitiligo; at least one in 100 British adults is affected. It affects white skins as much as black and Asian ones, and men as much as women, with the average age of onset just 20 years old.
The condition led the popstar Michael Jackson into the life of a recluse. He ventured out only under heavy layers of thick white make-up, or wearing masks to protect his fragile pale skin from the agony of sunburn. With no pigment, skin is extremely susceptible to burning. Other celebrity sufferers include chat show host Graham Norton, who is rarely seen without thick make-up, and Jon Hamm, the star of TV’s Mad Men.
Jennifer Viles, of charity the Vitiligo Society, says: “The effect of the condition can be very upsetting and people are treated differently; for some people it is considered legitimate grounds for divorce.”
Vitiligo is a so-called auto-immune condition, where the body’s immune system attacks itself — in this case, it attacks the pigment cells that produce melanin, which gives skin its colour and protects it from sunburn.
It can occur anywhere on the body and sometimes appears in symmetrical patches on each arm or leg. It can also remove pigment from hair, another problem which has affected Emma.
The condition is genetic, but it can also be triggered by stress; skin damage, such as severe sunburn; insect bites in susceptible people; or exposure to chemicals.
Indeed, Emma believes she inherited a tendency to it from her father, who has vitiligo on his arms and legs. She herself had a small patch of depigmented skin in the middle of her back which appeared when she was around 35, but believes it was the trauma of Genevieve being born with brain abnormalities that caused her vitiligo to spiral out of control.
Genevieve had to undergo repeated neurosurgery to relieve the pressure in her head and three months of intensive nursing before she could go home with her parents for the first time.
She is now seven and, along with her older sister Isabelle, fit and healthy and attending a mainstream school. But Emma continues to suffer from the condition triggered by that stressful time. “It’s hard to describe the emotional agony and anguish caused by a skin condition like this, and there is absolutely nothing you can do about it,” she says.
“My husband is always very tactful and if I don’t mention it he doesn’t say anything about it.
“But we live in a society totally obsessed by appearance. When people complain about wrinkles I think, ‘I would willingly swap the white patches on my face for any number of wrinkles’.
“Another problem is that without sunscreen, I could get horribly burned walking the few yards from Waterloo station to the skin clinic at St Thomas’ Hospital.
“I am not taking any medication at the moment. I just live with it, and I have to be very careful about staying out of the sun on holiday.”
To make matters worse, each of Emma’s daughters has a 50-50 chance of developing vitiligo, a prospect that she admits she finds “quite devastating”.
In the meantime, she plans to bring them up to have the self-esteem and confidence to deal with the problem if it arises, all the time hoping for a cure.
Despite the large number of sufferers, there is no particularly effective treatment for vitiligo other than steroid creams that dampen the condition for some people, through a mechanism which is not properly understood. However, long-term use of steroids can itself do further damage, such as skin thinning or stretching.
The alternative is regular exposure to specific wavelengths of ultraviolet light, which may occasionally stimulate some re-pigmentation, but has to be done every other day. Otherwise, sufferers are condemned to a condition that may have periods of inactivity before spreading further.
A new drug called Scenesse, currently at trial stage, may bring new hope. It is based on a compound called afamelanotide, which mimics the effect of melanocyte stimulating hormone, which is involved in the production of melanocytes — the skin pigment cells.
The drug, delivered as an implant about the size of a grain of rice, is put under the skin three or four times a year. In trials involving its use as a treatment for erythropoietic protoporphyria, a rare skin disease where people have no pigment at all, the drug was so successful that volunteers begged to stay on it when the experiment ended.
Doctors running studies in the US, France, Germany and Italy, involving 120 sufferers, are hopeful they are on the brink of applying for the first ever medical licence for a treatment specifically designed to cure vitiligo.
Pearl Grimes, professor of dermatology at the University of California, is one of the lead investigators in the Scenesse trial.
“We still need to analyse the results from the first group of patients and then test the drug in thousands more to establish it is definitely safe, but the results so far are very exciting,” she says.
Viktoria Eleftheriadou, a skin specialist researching the possibilities of different vitiligo treatments at Nottingham University, who has no connection with the Scenesse project, says: “The results in the small number of patients treated so far appear to have been good, and this treatment is frequently mentioned by specialist clinicians as being a very promising solution.”
Jennifer Viles is cautiously optimistic. “Vitiligo is not life threatening, but it does have a major psychological impact,” she says. “Until now, there has not been much interest in finding a new treatment that works. We are hoping for the best.” - Daily Mail