Rare disease causes an extreme sensitivity to sunlight

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When you have a rare disease called XP, you must take extreme precautions to protect your skin from the sun.

Everyone has sun-sensitive skin. It’s the reason we tan and sunburn. It’s why freckles, age spots, and skin cancers appear.

Some people are born more sun sensitive than others. People who have an extreme sensitivity to sunlight are born with a rare disease known as xeroderma (zer-o-der-ma) pigmentosum (pig-men-toe-sum). They must take extreme measures to protect their skin from ultraviolet (UV) light. Anything that emits UV light, including the sun and some lightbulbs, can damage their skin.

When people with xeroderma pigmentosum (XP) go outdoors during daylight, they need to cover up. Dermatologists recommend that they wear clothing which offers maximum protection from UV light. To protect their face and eyes, they should put on UV-protective sunglasses, goggles, or a face shield. Beneath this outer protective layer, they have on sunscreen, which is necessary should any skin become uncovered.

People who have XP need this extreme protection because their bodies cannot protect them from UV light. This happens because they inherit genes that prevent their bodies from repairing damage caused by UV ligh

XP dramatically increases the risk of skin cancer

Because the body cannot protect itself from UV light, a person’s risk of getting skin cancer skyrockets. It’s estimated that XP can raise the risk of getting skin cancer 10,000-fold.

Most children who have XP develop skin cancer before reaching 10 years of age. At an even younger age, these children can have pre-cancerous growths on their skin.

Skin cancers and pre-cancerous growths develop because it takes time to diagnose XP. You cannot look at a newborn and tell that the child has XP.

This disease tends to reveal itself when a child spends time outdoors. Some children develop a serious sunburn the first time daylight hits their skin. Not all children who have XP sunburn so easily.

For many children, the first noticeable sign of XP is many freckles on their skin, which usually develop before the child is 2 years old. As the child spends time in the sun, larger dark spots also appear. The skin will also feel extremely dry.

It’s these warning signs that give the disease its name. The word “xeroderma” means extremely dry skin and “pigmentosum” refers to the freckles and dark spots.

People who have XP may also develop eye problems. Sunlight can cause cancers to form on their eyes or eyelids. Cataracts and other eye problems may also arise.

Girl with XP: By 13 years of age, freckles blanket her skin and skin cancer appears on her leg.

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Getting an accurate diagnosis is essential

If you suspect that your child may have XP, it’s important to find out. Anyone who has XP needs extreme sun protection and expert medical care. You never outgrow XP.

To get an accurate diagnosis, people are usually referred to a board-certified dermatologist. If the diagnosis is XP, working with a dermatologist helps to:

  • Reduce the risk of getting skin cancers
  • Find and treat skin cancers early before the cancer spreads
  • Coordinate care with other health care professionals

Seeing other health care professionals is usually necessary because people who have XP can develop related health problems. These problems include possible troubles with eyesight, hearing loss, and spastic muscles.

Coping with all of this may sound difficult, but it hasn’t stopped Aimee Milota, a teenager who has XP. She’s an active teenager, living life to the fullest. You can read Aimee’s story at:

Living with extreme chronic skin cancer risk

Dermatologists are helping advance medical care for XP

To improve the lives of their patients with XP, dermatologists continue to study this disease. Their work has led to:

  • Improvements in sun protection
  • The discovery of medications that can reduce the risk of developing skin cancers
  • Insight into how skin cancer develops and can be prevented

This research can help everyone, not only their patients with XP. We all have sun-sensitive skin. Every year, millions of Americans develop skin cancer.

Sun protection helps everyone prevent skin cancer

While people who have XP require extreme sun protection, everyone can benefit from sun protection. For most people, skin cancer is preventable.

You can find out how to protect your skin at:

Prevent skin cancer

Additional information

Understanding xeroderma pigmentosum


Images
Image 1: Getty Images

Image 2: Used with permission of the Journal of the American Academy of Dermatology. J Am Acad Dermatol 2000;43:900-2.

References
American Academy of Dermatology. “Member to Member: Why was the XP-ERG formed?” Member-to-Member. March 2016.

Carruthers J. “Member making a difference: Kenneth Kraemer, MD.” Dermatol World. 2011; 21(5):44.

DiGiovanna JJ and Kramer KH. “Shining a light on xeroderma pigmentosum.” J Invest Dermatol. 2012; 132(3, part 2):785-96.

Kraemer KH and DiGiovanna JJ. “Forty years of research on xeroderma pigmentosum at the US National Institutes of Health.” Photochem Photobiol. 2015; 91(2): 452-9.

Lim HW and Hawk JLM. “Photodermatoses.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1341-3.

Rünger TM, DiGiovanna JJ, et al. “Hereditary disorders of genome instability and DNA repair.” Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008: 1315-9.