Nonuremic calciphylaxis associated with histologic changes of pseudoxanthoma elasticum

Area of Science:

• Vascular Medicine
• Dermatopathology
• Nephrology

Background:

• Calciphylaxis is a rare vascular disorder typically seen in end-stage renal disease (ESRD) patients.
• It involves medial calcification of small- and medium-sized vessels, leading to ischemic necrosis.
• Secondary hyperparathyroidism is a common comorbidity in ESRD-related calciphylaxis.

Observation:

• This report details a unique case of calciphylaxis in a patient without ESRD.
• The patient presented with primary hyperparathyroidism and alcoholic cirrhosis as potential contributing factors.
• Histopathologic examination revealed fragmentation and calcification of dermal elastic fibers, characteristic of pseudoxanthoma elasticum.

Findings:

• Calciphylaxis occurred in a nonuremic individual.
• Primary hyperparathyroidism and/or alcoholic cirrhosis were identified as likely etiologies.
• The case demonstrated concurrent calciphylaxis and pseudoxanthoma elasticum-like histopathologic changes.

Implications:

• This case expands the known clinical spectrum of calciphylaxis beyond ESRD.
• It highlights potential alternative causes, including primary hyperparathyroidism and alcoholic cirrhosis.
• The co-occurrence with pseudoxanthoma elasticum-like changes suggests potential shared pathomechanisms or a novel presentation.