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Nonuremic Calciphylaxis Associated With Histologic Changes of Pseudoxanthoma Elasticum

Fernandez, Kristen H., MD; Liu, Vincent, MD; Swick, Brian L., MD

The American Journal of Dermatopathology: February 2013 - Volume 35 - Issue 1 - p 106–108
doi: 10.1097/DAD.0b013e31826330db
Brief Report

Abstract: Calciphylaxis is a rare condition characterized by medial calcification of small- and medium-sized vessels that subsequently leads to ischemic necrosis. Calciphylaxis most often occurs in patients with end-stage renal disease and secondary hyperparathyroidism. We present a unique case of calciphylaxis in which the patient did not have end-stage renal disease. Instead, primary hyperparathyroidism and/or alcoholic cirrhosis were the more likely causes of her calciphylaxis. In addition, our case demonstrated not only calciphylaxis but also fragmentation and calcification of elastic fibers within the dermis, changes that are most often seen in pseudoxanthoma elasticum. This is the first reported case of calciphylaxis, to our knowledge, with histopathologic changes of pseudoxanthoma elasticum in a patient who is nonuremic.

Department of Dermatology, University of Missouri, Columbia, MO.

Reprints: Kristen H. Fernandez, MD, Department of Dermatology, University of Missouri, one hospital drive, Room MA 111, Columbia, MO 65212 (e-mail:

The authors declare no conflicts of interest.

© 2013 by Lippincott Williams & Wilkins.