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Dentistry
and PXE
By Joseph O'Brien Daley, DMD
What is PXE and what are the dental implications? PXE (pseudoxanthomaelasticum)
is an inherited disorder which causes excessive calcification of connective
tissue with specific attention to the elastic component. Therefore,
thepathological changes primarily occur in the elastic tissues such
as the skin, eyes,cardiovascular and gastrointestinal systems. Inheritance
is thought by some to be autosomal recessive, but families with an apparent
autosomal dominant pattern have
also been described.
The clinical presentation of
PXE often includes the following:
Persistent raised, yellowish-white plaque-like rash most often on the
neckbut may occur anywhere on the skin.
Lower lip: beds or streaks of whitish-yellow patches along thelabial
mucosa which can often be misinterpreted as Fordyce spots.
The diagnosis of PXE rests on the clinical signs and is confirmed by histologic examination. The incidence of PXE in the general population is very low. However, the disease can be devastating. The hope is through early detection of the clinical signs, diagnosis and treatment can be started early in life.
For the dental profession the key is early recognition and referral to a dermatologist, ophthalmologist and cardiologist for further evaluation.
Reference: Goette, Detlef K., and Carpenter, William M.: The Mucocutenous
Marker of Pseudoxanthoma Elasticum, Oral Surgery, Oral Medicine, Oral
Pathology. 51(1):68-72,1981.
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