An unusual site for calciphylaxis: a case report.

Area of Science:

• Vascular Medicine
• Dermatology
• Endocrinology

Background:

• Calciphylaxis is a rare condition causing arterial calcification, thrombosis, and tissue necrosis.
• It is typically associated with end-stage renal failure, though not always present.

Observation:

• An 83-year-old female with primary hyperparathyroidism and mild hypercalcemia treated with bisphosphonates presented with a rapidly progressing necrotic vulval lesion.
• Biopsy confirmed calciphylaxis, showing extensive necrosis, abscesses, and calcified, thrombosed vessels.

Findings:

• This case presents calciphylaxis in a patient without end-stage renal failure, linked to primary hyperparathyroidism.
• The vulval lesion appeared 9 months after initiating bisphosphonate therapy, raising questions about its potential role in pathogenesis.
• The patient had decreased serum parathyroid hormone and calcium levels after treatment.

Implications:

• This case expands the differential diagnosis for necrotic vulval lesions, suggesting calciphylaxis as a possibility beyond malignancy.
• It prompts further investigation into the role of bisphosphonates in calciphylaxis, particularly in non-uremic patients.
• Understanding the pathogenesis of calciphylaxis remains crucial for effective management.