PXE Discovery and Definition

Although PXE had been around for a very long time it was only in August 1896 at the third International Congress of Dermatology in London that Jean Darrier from Paris accurately described PXE.

The centenary of this important event was duly commemorated in High Wycombe, some 30 miles from London, on 4th August 1996 when a conference and seminar on PXE was held, attended by over 100 PXE, together with their families. Attendees came from as far apart as the Channel Islands, Scotland, Ireland, Wales and the continent of Europe. The principle speaker was Dr Kenneth Neldner from Texas.

PXE is a hereditary disorder in which the elastic fibres that normally occur in the skin, eyes and cardiovascular system gradually become calcified and cause characteristic symptoms in each area.

It is estimated that about 1 in 25,000 people in the world have PXE but recent indications are that it is more common. Unfortunately, accurate figures are difficult to obtain. For unknown reasons, there are approximately twice as many females as males with PXE, the average age of onset being about 13 years.