11 year old boy has Xeroderma Pigmentosum, can't stay under the Sun, lives in permanent shade: Know all about it

3 months ago 22

We all have learnt about the benefits of sunlight and how lack of it affects our body's ability to synthesize vitamin D and use it for bodily functions. But, what if your health condition does not allow you to stay under the Sun?
For 11 year old Pol Dominguez summer vacations mean staying indoors to avoid ultraviolet radiation. Reason: Sunlight could be deadly for him.
The boy from Spain has a rare condition called

Xeroderma Pigmentosum

that affects his skin and eyes.


Pol Dominguez

(Image: Reuters)


What is Xeroderma Pigmentosum?

Xeroderma Pigmentosum (XP) is a rare

genetic disorder

that severely affects how individuals interact with sunlight. People with XP have a genetic mutation that impairs their ability to repair damage caused by ultraviolet (UV) light exposure. This condition is inherited in an autosomal recessive pattern, meaning a child must inherit two defective copies of the gene (one from each parent) to develop XP.
Living with this condition means avoiding daylight hours as it poses significant risks. The hallmark of XP is extreme sensitivity to

UV radiation

. Even brief exposure to sunlight can lead to severe sunburns, blistering, and skin damage. This sensitivity extends to artificial sources of UV light, including fluorescent bulbs and tanning beds, which are also problematic for individuals with XP.

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Beyond the immediate discomfort of sunburns, XP patients often develop freckles and irregular patches of pigmentation on sun-exposed areas of the skin. These patches can darken or lighten over time, adding to the distinctive appearance associated with the condition. Moreover, the cumulative damage from UV exposure dramatically increases the risk of developing skin cancers at a young age.
Patients are unable to repair their DNA from solar damage, which puts them at high risk of developing cancer. Basal cell carcinoma, squamous cell carcinoma, and melanoma are all more prevalent in individuals with XP compared to the general population.
Individuals must wear

protective clothing

, including hats, long sleeves, and sunglasses with UV-blocking lenses. Specialized sunscreens with high SPF and broad-spectrum protection are essential, and reapplication is crucial throughout the day. Some individuals with XP may also need to modify their daily routines to minimize exposure during peak sunlight hours or seek indoor environments with UV-filtered windows.
(With inputs from Reuters)

Article From: timesofindia.indiatimes.com
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