Q: My cousin's two teenage children have been diagnosed with multiple skin cancers and advised to remain completely indoors. Could you explain what disease or disorder they might be having?

A: Your cousin’s children are most likely the unfortunate sufferers of Xeroderma Pigmentosum (XP). This is a very rare skin disorder which makes the affected infants and children extremely over sensitive to sunlight, mainly the ultraviolet radiations. XP is a genetically mediated disease. The earliest clinical signs are sudden reddening of the exposed areas of the body upon exposing to sun. On subsequent prolonged sun exposures, these reactions continue getting more intense leading to repeated acute sun burn reactions.

The continuous skin cell damage in XP sufferers make them extremely vulnerable to develop various types of skin cancers.

Eye problems are the second commonest issue amongst the XP sufferers affecting almost 75 per cent of them. Their eye complaints mainly include painfully sensitive eyes to the sun (photophobia), easy irritability of the eyes with blood shots and cloudiness (conjunctivitis) and rarely appearance of non-cancerous and cancerous growth on the eyes. It can also include spasticity, poor coordination, deafness, developmental delays and short stature. However, the neurological problems do manifest themselves at a bit later age comparing the skin and eye problems.

There is no cure for XP, but certain basic guidelines are mandatory to be followed for the sufferers which include:

•A total protection from UV radiations from the sun and unshielded fluorescent lamps.

•Outdoor activities must be restricted to nighttime. Wear protective clothing

• Frequent skin examinations by experienced dermatologist and a specialist neurologist.

Dr Ikramullah Nasir is specialist dermatologist and medical director at Dermacare Dubai. Our fantastic panel of renowned experts is available to answer all your questions related to fashion, well-being, nutrition, finance and hypnotherapy. Email your queries to friday@gulfnews.com.

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