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By GARDY CHACHA
A disease that makes use of darkness as real medicine
Xeroderma Pigmentosum (XP), is a genetic condition characterised by an extreme sensitivity to light or the ultraviolet (UV) rays from sunlight
This condition mostly affects the eyes and areas of skin exposed to the sun.
The signs of xeroderma pigmentosum usually appear in infancy or early childhood.
Many affected children develop severe sunburn after spending just a few minutes in the sun. The sunburn causes redness and blistering that can last for weeks.
By the age of two, almost all children with xeroderma pigmentosum develop freckling of the skin even if they were to be kept in darkness, which is the only sure way of avoiding its effects.
Exposed areas (such as the face, arms and lips) usually develop leopard-like marks when a baby is still small because it’s difficult to avoid light.
In affected individuals, exposure to sunlight often causes dry skin (xeroderma) and changes in skin colouring (pigmentation). This combination of features gives the condition its name ¬— xeroderma pigmentosum.
People with xeroderma pigmentosum have a greatly increased risk of developing skin cancer. Without sun protection, about half of children with this condition develop their first skin cancer by the age of ten.
OTHER ABNORMALITIES
Most people with xeroderma pigmentosum develop multiple skin cancers during their lifetime.
These cancers occur most often on the face, lips and eyelids. Cancer can also develop on the scalp, in the eyes, and on the tip of the tongue.
Studies suggest that people with xeroderma pigmentosum may also have an increased risk of other types of cancer, including brain tumours.
The eyes of people with xeroderma pigmentosum are also painfully sensitive to UV rays from the sun.
If the eyes are not protected from the sun, they may become bloodshot and irritated, and the clear front covering of the eyes (the cornea) may become cloudy. In some people, the eyelashes fall out and the eyelids may be thin and turn abnormally inward or outward.
In addition to an increased risk of eye cancer, xeroderma pigmentosum is associated with non-cancerous growths on the eye. Many of these eye abnormalities can impair vision.
About 30 percent of people with xeroderma pigmentosum develop progressive neurological abnormalities in addition to problems involving the skin and eyes.
These abnormalities can include hearing loss, poor coordination, difficulty walking, movement problems, loss of intellectual function, difficulty in swallowing and talking, and seizures.
When these neurological problems occur, they tend to worsen with time. The only sure way to prevent being affected is by living in darkness.
Some people call XP ‘Life in Darkness’ disease.
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