A Case of Pseudoxanthoma Elasticum - like Papillary Dermal Elastolysis

Lee, Ga Young; Choi, Hae Young; Myung, Ki Bum

Abstract

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an unusual acquired elastolytic disorder that clinically resembles PXE but shows partial or complete loss of the elastic network in the papillary dermis. PXE-like PDE is characterized by the multiple yellowish nonfollicular papules, small in diameter, on the antecubital fossae, flexor aspect of the forearms, supraclavicular region, sides of the neck, and lower abdominal area. The multiple papules coalesce to form plaques with a cobble stone appearance resembling "plucked chicken skin". No systemic involvement has been noted in this disorder. Histopathologically, Hematoxylin-eosin stain shows nearly normal finding, but elastic stain shows partial or complete loss of elastic fibers focally in the papillary dermis. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis with typical clinical and pathological features.