Pseudoxanthoma elasticum, PXE, is an inherited disorder that causes select elastic tissue in the body to become mineralized, that is, calcium and other minerals are deposited in the tissue. This can result in changes in the skin, eyes, cardiovascular system and gastrointestinal system. Changes in the skin are usually the earliest sign of PXE, and lead to the definitive diagnosis.
Estimates of the incidence of PXE range from 1 in 100,000 people to 1 in 25,000 people. However, the true incidence of PXE is not known in any population.
Dentistry
PXE may appear as follows:
- Persistent raised, yellowish-white plaque-like rash most often on the neck but may occur anywhere on the skin.
- Lower lip: beds or streaks of whitish-yellow patches along the labial mucosa, which can often be misinterpreted as Fordyce spots, which often occur in all people.
For the dental profession the key is early recognition and referral to a dermatologist, ophthalmologist and cardiologist for further evaluation.
PXE International provides referrals to specialists.
