Hematoxylin-eosin–stained sections showed focal and slight thickening of collagen in the papillary dermis. Elastic fibers did not appear calcified or fragmented. Sections stained for elastin revealed focal loss of superficial dermal elastic fibers in the central portion of the biopsy specimen.
In 1992, Rongioletti and Rebora1 described 2 patients with lesions that clinically resembled PXE but with a total loss of the elastic network in the papillary dermis. Since then, only a few cases of this unusual acquired elastolytic disorder, termed PXE-like papillary dermal elastolysis, have been reported in the literature.2-6 Pseudoxanthoma elasticum–like papillary dermal elastolysis mainly affects women in late adulthood (60-80 years of age). Cutaneous lesions clinically resemble PXE. Multiple yellowish nonfollicular papules, small in diameter, coalesce to form patches with a cobblestone appearance resembling "plucked chicken skin." They are symmetrically distributed over the antecubital fossae, flexor aspect of the forearms, supraclavicular region, sides of the neck, and lower abdominal area. No systemic involvement has been noted in this disorder. The skin lesions are mostly asymptomatic but slowly increase in number over time.