Beauty Industry Bulletins

How is xeroderma pigmentosum treated

There is currently no cure for xeroderma pigmentosum (XP), a genetic condition that does not repair DNA damaged by ultraviolet (UV) light. For those with the condition, options for treating the disorder range from rigorous prevention of UV exposure to removing skin cancer to treating related ocular and neurological abnormalities.

This article discusses home and lifestyle remedies, over-the-counter treatments, prescription medications, and specialist procedures for treating and managing xeroderma pigmentosum.

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Home remedies and lifestyle

Treatment at home is all about carefully avoiding the sun and staying away from fluorescent lamps, which also pose a UV risk. Those with XP are advised to do the following:

  • Stay indoors during the day and take part in outdoor activities after dark.
  • Protect the skin by covering it with non-porous clothing such as long-sleeved shirts, long pants, high collars, and wide-brimmed hats.
  • Apply sunscreen with a sun protection factor (SPF) of at least 50 on every exposed skin – the higher the sun protection factor, the better.
  • Protect your eyes with UV-blocking all-round sunglasses.
  • Use a UV-blocking face shield.
  • Use a UV light meter to check your home or school for UV levels and remove UV rays from the surrounding area if possible.
  • Apply UV blocking film to all windows in your home, school, workplace, or car as harmful UV rays can penetrate through glass.
  • Avoid cigarette smoke, which is also known to damage DNA.
  • Check your skin for new spots or lesions that could be cancerous.

Over-the-counter therapies

In addition to buying preventative items from the drugstore, like sunscreen and sunglasses or pain relievers for the pain of sunburn, you can also get vitamin D, which is needed for building healthy bones.

Normally, the body makes vitamin D when it is exposed to the sun. Since people with XP have to avoid the sun, taking vitamin D supplements is necessary to prevent vitamin D deficiency.

A dietary supplement like Heliocare, which contains Fernblock, an extract of the polypodium leucomotos fern, has been shown to help repair damage caused by UV radiation. Fernblock is also used in topical sunscreen preparations.

Also, for those affected by dry eyes from xeroderma pigmentosum, moisturizing eye drops can be used to help keep the cornea moist.

Recipes

In some severely affected patients, the prescription drug isotretinoin, an oral drug based on vitamin A, can reduce the number of skin tumors that develop.

The downside is that this drug has been associated with serious side effects, such as: High levels of cholesterol and triglycerides in the blood, the possibility of causing defects in developing fetuses, and painful calcium deposits on tendons and ligaments.

Operations and specialist procedures

For those with XP, operations to remove precancerous and cancerous lesions are a big part of surgical treatment. This means that ongoing screening is also an important part of the treatment process.

Those with XP should follow this screening routine:

  • Every three to six months, have your skin carefully examined by a professional trained in detecting skin cancer.
  • As often as possible, get a family member who knows the signs of skin cancer in people with XP to examine any areas that may be difficult to see.
  • See an ophthalmologist (a doctor who specializes in eye conditions) regularly for eye exams.
  • Have your mouth checked regularly by a dentist who can spot any lesions.
  • See your GP for regular neurological exams.

Surgery for XP patients is a mainstay of treatment. Possible surgical interventions can be the following:

  • Removal of premalignant (precancerous) lesions in patients with XP, e.g. B. by freezing with liquid nitrogen
  • Using topical creams such as Efudex (topical 5-fluorouracil) or Aldara (imiquimod) to treat larger areas of damaged skin
  • Surgical excision or electrosication of suspicious lesions for small areas in need of treatment on the extremities and trunk
  • Dermatome shave and dermabrasion when larger areas of skin need to be treated
  • Mohs’ micrographic surgery for the treatment of deep skin cancer in areas that require tissue protection, such as: B. on the face
  • Retransplantation or replacement of large areas of skin with sun-protected skin
  • X-ray therapy for inoperable cancers

XP patients can also have eye problems. Here are some treatment options when these occur:

  • Soft contact lenses can be prescribed to protect the surface if the eyelids begin to rub against the eyes and cause irritation.
  • All cancers of the eyelids, conjunctiva (the membrane that covers the whites of the eye), or the cornea (clear dome on the front of the eye) can be surgically removed.
  • Corneal transplantation may be considered for UV-induced eye damage. However, success here may be limited as drugs needed to prevent graft rejection can cause additional skin cancers.
  • Topical chemotherapy drugs can be used for all cancers of the conjunctiva or sclera (the white part of the eye).

In addition, some XP patients may have neurological problems. Treatment for this includes:

  • Use of hearing aids by people with associated hearing loss that tends to get worse over time
  • Using cochlear implants to help some XP patients with severe hearing loss
  • Help through special programs in schools for children with learning difficulties from XP, such as special needs education and occupational therapy

summary

Treatment of skin lesions and ocular or neurological symptoms remains limited in patients with XP. Preventive measures such as wearing protective clothing and sunscreen as well as avoiding UV exposure are the mainstays. The early removal of any precancerous or cancerous lesions that may appear is central here and relies on frequent screening measures.

A word from Verywell

While treatment options for XP are currently limited to prevent skin lesions or eye symptoms, the hope for the future is that a genetic treatment will be developed that will allow patients with XP to enjoy the light without any consequences.

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