Pseudoxanthoma elasticum-like papillary dermal elastolysis
Pseudoxanthoma elasticum-like papillary dermal elastolysis
Definition
Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare, acquired, idiopathic dermal tissue disorder characterized by numerous, asymptomatic, 2-3 mm, yellowish, non-follicular papules that tend to converge into cobblestone-like plaques which are distributed symmetrically over the posterior neck, supraclavicular region, axillae, and sometimes abdomen. Unlike PXE, these skin lesions show select elimination (absence or marked loss) of elastic fibers in the papillary dermis and there is no systemic involvement.
Also known as PXE-like papillary dermal elastolysis, PXE-PDE — per MONDO