TREATMENT OF XERODERMA PIGMENTOSUM 

Edited by, SOWNDHARYA

Read about Xeroderma pigmentosum, Causes, Symptoms, Effects and diagnosis

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Standard Therapies:

PREVENTION:

• Rigorous sun (UV) protection is necessary beginning as soon as the diagnosis is suspected to prevent continued DNA damage and disease progression. Individuals with XP should avoid exposing the skin and eyes to ultraviolet radiation. This can be done by wearing protective clothing such as hats, hoods with UV blocking face shields, long sleeves, pants and gloves. High Sun-protective factor (SPF) sunscreens, UV-blocking glasses with side-shields and long hair can also provide protection.

• The XP patients surroundings should be tested for levels of UV using a UV light meter. The meter can help identify areas of increased UV and sources of damaging UV can be eliminated from the environment.

• Vitamin D is an essential vitamin, which helps maintain healthy bones. Vitamin D is manufactured by the interaction of UV with the skin. Since people with XP avoid UV, oral dietary supplements may be taken as needed.

DERMATOLOGIC CARE:

• The skin including the scalp, lips, tongue and eyelids should be examined by the dermatologist every 6 to 12 months to detect pre-cancerous and cancerous lesions.

• Individuals should also undergo routine eye exams by an ophthalmologist. The eyelids should be examined for ectropion (drooping and sagging), entropion (inward rotation, which may cause irritation) and pterygia/pinguecula (benign growth on the surface of the eyes)

NEUROLOGIC EXAMINATIONS:

Basic neurology examinations including measuring the occipital frontal circumference (to determine the presence of microcephaly) and accessing for the presence of deep tendon reflexes, should be part of the routine care of an XP patient. Hearing exams should be done on a regular basis to access for early onset hearing loss, which is an indicator of XP with neurologic disease.

TREATMENT OF SKIN CANCERS:

Small, premalignant skin lesions, (example: actinic keratosis) can be treated by freezing with liquid nitrogen. For larger areas of damaged skin, topical creams such as 5-fluorouracil or imiquimod may be applied; dermatome shaving and dermabrasion have been used for larger areas of skin. Small skin cancers on the trunk and extremities can be treated with electrodesiccation and curettage, or surgical excision.

TREATMENT OF XP EYE ABNORMALITIES:

Lubricating eye drops used frequently keep the cornea moist and protects against the inflammatory effects of dry eye. Soft contact lenses can be worn to protect against mechanical trauma caused by deformed eyelids.

TREATMENT OF XP NEUROLOGIC ABNORMALITIES :

Neurology abnormalities are associated with increased high-frequency sensory-neural hearing loss. The hearing loss is progressive and can be treated with hearing aids. Cognitive delays can be seen in childhood and special education classes, physical and occupational therapists along with UV safe accommodations at school are very helpful for XP children. As they get older, people with XP neurologic disease also experience increasing ataxia, dysphagia and dysarthria as the condition progresses.