Non-uraemic calciphylaxis successfully treated with pamidronate infusion

Abstract

Calciphylaxis is a rare and potentially fatal disease that affects the subcutaneous layer of the skin. It is a calcific vasculopathy induced by a systemic process that causes occlusion of small blood vessels. The mortality rate for individuals diagnosed with calciphylaxis is estimated between 52% and 81% with sepsis being the leading cause of death. Uraemic calciphylaxis and its known effective treatments are well documented in the literature. Unfortunately, there is no known effective treatment for non-uraemic calciphylaxis. Most of the current treatments for non-uraemic calciphylaxis are derived from uraemic calciphylaxis treatment protocols. We report a case of a 75-year-old female with calciphylaxis on the right lower extremity who was successfully treated with four pamidronate infusions in addition to local wound care. This case represents a non-uraemic calciphylaxis wound successfully treated with pamidronate infusions and standard wound care, and suggests that IV pamidronate can be an effective treatment option.

Keywords: calcific arteriolopathy; leg ulcer; non-healing wound; sodium thiosulphate; vascular calcifications.

Figure 1

Initial presentation of right lower extremity wounds—Oedematous with multiple discoloured crusted lesions with violaceus borders on the posterior, medial, and lateral aspect of the right distal leg

Figure 2

One month after initial presentation. The wound had worsened with an eschar developed over the crusted lesion along increased pain

Figure 3

Right lower extremity wounds 3 months after initial presentation. The discoloured lesions had transformed into extremely tender reticulate purpura with black eschar and violaceous borders

Figure 4

Histology of right lower extremity wound. The specimen shows small vessels in the interlobular septa and at the periphery of a lobule that show some calcific deposits in the vessel walls. One of the superficial dermal‐dilated small vessels contains a small amount of fibrin thrombus. A von Kossa stain for calcium was carried out and fine granular calcific deposits were found in the vessels in the dermal subcutaneous layer and in smaller capillary size vessels in the superficial subcutaneous tissue

Figure 5

Four months since treatment started. Two weeks status post the first pamidronate infusion. The eschars had almost completely resolved with improvement in ulcer size and wound base appearance

Figure 6

After third infusion of pamidronate. The eschars and fibrotic wounds base had completely resolved. Epithelisation was noted with improvement in wound size and pain

Figure 7

After fourth pamidronate infusion. Wounds had completely closed

Figure 8

One month since the last pamidronate infusion. The wound remained closed

Figure 9

Suggested initial treatment guideline for non‐uraemic calciphylaxis (NUC). Once etiology is determined, treatment of the underlying condition is recommended