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Pseudoxanthoma Elasticum-Like Papillary Dermal Elastolysis: A Single Case Report.

Venus Valbuena¹, Dalal Assaad^1,2, Jensen Yeung^1,2,3

¹1 University of Toronto Faculty of Medicine, Toronto, ON, Canada.

Journal of Cutaneous Medicine and Surgery

|March 11, 2017

Summary

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare skin condition in older women. Histopathology confirmed PXE-PDE, distinguishing it from papular elastorrhexis despite initial consideration.

Keywords:

elastic tissue disorders pseudoxanthoma elasticum–like papillary dermal elastolysis

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Area of Science:

Dermatology
Pathology
Histopathology

Background:

Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare acquired elastolytic disorder affecting postmenopausal women.
Characterized by papules on the neck and supraclavicular regions, it is histologically distinct from inherited pseudoxanthoma elasticum.

Observation:

A 64-year-old woman presented with asymptomatic, soft, yellow papules on her neck.
Histopathology revealed absent elastic fibers in the papillary dermis, with initial consideration of papular elastorrhexis.

Findings:

Clinicopathologic correlation confirmed PXE-PDE, characterized by a significant loss of elastic fibers in the papillary dermis.
Intralesional corticosteroids were ineffective in treating the condition.

Implications:

Accurate histopathology and clinicopathologic correlation are crucial for differentiating PXE-PDE from similar fibroelastolytic disorders.
This case highlights the diagnostic challenges and treatment considerations for PXE-PDE.