34806 Pseudoxanthoma elasticum-like papillary dermal elastolysis: A histopathologic diagnosis

Abstract

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare acquired dermatologic disorder of the elastic tissue that mimics genetic pseudoxanthoma elasticum (PXE). PXE-PDE is characterized clinically by flesh-colored to yellow papules that often coalesce into cobblestone plaques on flexural sites predominately in postmenopausal women. PXE-PDE can be distinguished from true PXE on a histopathologic basis only, although the clinical presentation of PXE-like lesions without associated systemic complications makes a diagnosis of PXE-PDE more likely. The pathogenesis of PXE-PDE is unclear and has been suggested to be multifactorial, involving intrinsic, extrinsic, and genetic factors. Treatment of this disease has proven to be difficult, with various treatments having less than adequate outcomes. To our knowledge, there have been around 60 cases of PXE-PDE reported in the literature. Here, we present a case of pruritic PXE-PDE of the neck and antecubital fossae with intent to raise awareness of the most common clinical and histopathologic features.