Wilt u grote letters klik dan hier

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.

 

---------------------------------------------------------------------------------------------

Deze publicatie vervangt geen medische hulp
consulteer uw arts
--------------------------------------------------------------------------------------

 

Published by PXE International, Inc.
23 Mountain Street, Sharon, MA 02067
Voice: 781-784-3817
Fax: 781-784-6672
e-mail - info@pxe.org
website - www.pxe.org

Privacy Policy Disclaimer Funding
Maintained by PXE International.
Contact Webmaster.
Copyright © 2002-2005 PXE International, Inc. All rights reserved.