Prophylaxis
In order to be able to protect oneself from UV radiation, UV-impermeable protective clothing and sun protection agents help. In addition, glasses or a face mask with UV protection should be worn. The best way to avoid sunlight is to change the day-night rhythm, which should be done in childhood (moonlight children).
It has a considerable effect on later life and career choices. Prophylaxis of new skin tumors can be attempted by taking retinoids such as isoretinoin or aromatic retinoid. Retinoids are related to vitamin A (retinol). However, the dose has to be much higher than with usual therapies, which is why this drug therapy is often not tolerated.
Prognosis
The state of health is deteriorating increasingly. The risk of malignant skin tumors is 2000 times higher, so that the first skin tumor develops on average at the age of 8 years. Patients often die of malignant tumors (malignancies) that spread metastases before the age of three. However, there are also patients who have reached the sixth decade of life. Only consistent UV protection improves the course of the disease.
Summary
Xeroderma pigmentosum is a rare, autosomal recessive hereditary disease. Defective DNA repair mechanisms result in unresolved DNA damage, which leads to cell, tissue and organ damage. Life expectancy is shortened.
All articles in this series: