The Girls With Too Much Skin

The girls with too much skin, Wednesday 19th March 9pm on Five


“They looked like something from space - like an alien"

Lucy Betts, at eighteen years old, is the oldest surviving harlequin ichthyosis sufferer in Britain. Her survival is down to a life-saving ritual of scrubbing, bathing and creaming to keep the skin growth under control.

Tonight, in a documentary to be shown at 9pm on Five, Lucy and sister Hannah, who also suffers with the condition, will show how they have to live in a sterile environment and painfully exfoliate twice a day just to survive.

Many sufferers die within days of birth due to feeding issues, bacterial infection and/or respiratory diseases. But nowadays we are seeing more survivors with better neonatal care and treatments developing all the time.


A scaly suit of skin armour

Dana and Lara Bowen are also shown on tonight’s show, this is a photo of Dana.What is Harlequin Ichthyosis (HI)Harlequin Ichthyosis is a very rare, incurable, genetic skin condition. In it, the skin grows far faster than normal and dries out to form hard diamond shaped plaques. The whole body becomes completely encased in a very thickened, scaly 'suit of skin armour'. The skin becomes very tight causing severe restriction of movement of the arms and legs and in particularly, the fingers and toes. The face is deformed, the eyelids are inverted, the ears are bound down, and the mouth is very tight.

Research has been ongoing and the genetic cause of Harlequin Ichthyosis has recently been discovered by a team at Barts and the London, Queen Mary's School of Medicine and Dentistry. Mutations in the ABCA12 gene cause harlequin ichthyosis. The outcome was discovered after a seven year research program looking at children and families with the condition in Japan, USA and the UK.

It is hoped that now the mutation in the gene is known, new treatments can be offered to treat the condition as sufferers lives are constantly under threat from infection. It is also hoped that the discovery will lead to the possibility of prenatal screening.


"Just not compatible with living."

In a new documentary to be shown on Channel 5, Lucy and Hannah's father Clive tells of his shock at his daughters' appearance at birth, saying that they looked "like something from space - like an alien", and admitting that he did not expect them to live. Their paediatrician, Professor John Harper, feels similarly, saying that sufferers of the rare condition - only six cases are known in the UK - are "just not compatible with living."

The documentary follows the Betts and the Bowens, the bonds that the two families have developed in the face of their children's illness, and the insights that genetic analysis is giving into the disease.



Link to Telegraph's video portal - 4 minute video. Be warned, graphic images http://www.telegraph.co.uk/portal/ttv/news.jhtml?bcpid=1365202579&bclid=1155306408&bctid=1459170187
Photo 1 Lucy Betts
Photo 2 Dana Bowen
Photo 3 'The Harlequin sisters'