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Pseudoxanthoma elasticum-like papillary dermal elastolysis; A report of two cases and a literature review.

Dermatol Online J

October 2024

Division of Dermatology, Department of Medicine, School of Medicine, The University of Jordan, Amman, Jordan.

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a rare, benign, acquired, gradually-developing chronic elastic tissue disorder that almost exclusively affects post-menopausal women. It is essential to recognize this disease as it mimics the inherited pseudoxanthoma clinically. The pathophysiology behind this disease is multifactorial; it includes intrinsic skin aging, ultraviolet radiation exposure, and genetic components.

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Article Synopsis
  • * The condition usually appears during the teenage years and is primarily caused by mutations in specific genes, with most cases being sporadic or recessive.
  • * Recent research has identified several disorders that display PXE-like symptoms or microscopic features, prompting a discussion on the potential mechanisms that could cause this overlap.
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Ectopic calcification in the cardiovascular system adversely affects life prognosis. DBA/2 mice experience calcification owing to low expression of Abcc6 as observed in pseudoxanthoma elasticum (PXE) patients; however, little is known about its characteristics as a calcification model. In this study, we explore the suitability of a DBA/2 sub-strain as a PXE-like tissue calcification model, and the effect of a bisphosphonate which prevents calcification of soft tissues in hypercalcemic models was evaluated.

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