[Pseudoxanthoma elasticum-like papillary dermal elastolysis: A case report]

Area of Science:

• Dermatology
• Pathology
• Rare Diseases

Background:

• Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare condition.
• It clinically resembles pseudoxanthoma elasticum (PXE).

Observation:

• A 77-year-old woman presented with a 4-year history of asymptomatic papular eruption.
• The eruption was located on the axillae, chest, neck, and inguinal fold.
• Histology revealed papillary and mid-dermal elastolysis without elastorrhexis.

Findings:

• PXE-PDE is an acquired condition typically affecting women over 60.
• It is differentiated from PXE by late onset, lack of systemic symptoms, and specific histological findings.
• Histological examination shows reduced or absent elastic fibers in the papillary and mid-dermis.

Implications:

• Accurate diagnosis of PXE-PDE avoids unnecessary investigations.
• Understanding PXE-PDE aids in patient reassurance.
• Potential physiopathology may involve skin aging, elastogenesis, and UV exposure.