Abstract
Introduction: Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an acquired, localized elastic tissue disorder without systemic involvement that clinically presents as soft, yellow papules coalescing into cobblestone plaques that resemble PXE. It typically affects postmenopausal women and most commonly involves the neck followed by the supraclavicular region, axilla, forearms, and abdomen. An 82-year-old woman presented with pruritic papules and cobblestone plaques with a yellow hue located on the upper back, shoulders, upper chest, and upper arms; these lesions first appeared at 40 years of age, and the patient denied a family history of similar lesions. Two punch biopsies were obtained from involved and uninvolved skin. Histopathologic examination of involved skin revealed wedge-shaped focal fibrosis in the dermis; Verhoeff-van Gieson stain demonstrated relative decrease in elastin content within the papillary dermis compared to uninvolved skin. The involved skin showed no evidence of fragmented, clumped, or calcified elastic fibers, and fundoscopic exam did not identify angioid streaks, excluding PXE. We present a case of PXE-like PDE, one of several overlapping disorders that comprise the group known as fibroelastolytic papulosis. This report aims to highlight a mimicker of PXE, decrease unnecessary workup for this group of disorders that lacks systemic associations, and aid in histopathologic classification.
Financial Disclosure:
No current or relevant financial relationships exist.