Korean J Dermatol.  2011 Jan;49(1):53-57.

Pseudoxanthoma Elasticum Presenting as Localized Skin Lesion without Systemic Involvement

• Jang MS
• Kang DY
• Kang JS
• Baek JW
• Kim ST
• Suh KS

Abstract

Pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic tissue and it has many systemic manifestations. PXE is a systemic disorder that most markedly affects the elastic tissues in the skin, retina and blood vessels. Defects in the ABCC6 gene lead to calcification of elastic tissue. We herein report on two cases of pseudoxanthoma elasticum that presented with focal involvement on the skin of the neck without marked wrinkling.

Keyword

ABCC6 gene; Classification; Pseudoxanthoma elasticum

MeSH Terms

Blood Vessels
Elastic Tissue
Neck
Pseudoxanthoma Elasticum
Retina
Skin