Pseudoxanthoma elasticum-like papillary dermal elastolysis: a large case series with clinicopathological correlation

Area of Science:

• Dermatology
• Histopathology
• Medical Research

Background:

• Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is a rare acquired elastic tissue disorder.
• Fewer than 20 cases have been previously reported in medical literature.

Purpose of the Study:

• To report a case series of 17 patients with PXE-like PDE.
• To discuss the clinicopathological correlation of this condition.

Main Methods:

• Prospective collection of 17 cases of PXE-like PDE.
• Evaluation of demographic, clinical, and histopathological features.

Main Results:

• The study included 17 female patients with a mean age of 61.8 years.
• Common sites of involvement were the neck, supraclavicular region, and axilla.
• Histopathology revealed loss of elastic fibers in the papillary dermis and presence of melanophages, with no systemic involvement observed.

Conclusions:

• PXE-like PDE is characterized by selective elastic tissue loss in the papillary dermis and melanophages, potentially due to photodamage.
• The condition may be underdiagnosed, and awareness can prevent unnecessary investigations for patients.
• Accurate diagnosis relies on clinicopathological correlation, emphasizing the need for elastic tissue stains.