Calciphylaxis is increasing in incidence (5.7 per 10 000 chronic hemodialysis patients in 2011), and is most commonly associated with chronic renal failure, hemodialysis, and secondary hyperparathyroidism. There are also many cases of "nonuremic" or "nontraditional" calciphylaxis, which can occur in the setting of liver disease, diabetes, warfarin use, use of calcium-based phosphate binders, systemic corticosteroid use, solid organ malignancies, systemic lupus erythematosus, and Crohn disease. Other risk factors include female sex, obesity, Northern European descent, and hypoalbuminemia.
Notably, warfarin-associated nonuremic calciphylaxis tends to occur about 2.5 years after warfarin initiation on the lower extremities, does not have associated calcium abnormalities, and appears to have a more favorable prognosis than calciphylaxis associated with renal failure states.
Early lesions are extremely painful, violaceous retiform patches and plaques, classically on fat-bearing areas such as the thighs, buttocks, or abdomen. This is followed by necrosis, ulcers, eschar formation, and possibly gangrene. Mortality from calciphylaxis is high (60%-87%) and is largely secondary to sepsis from large, nonhealing ulcers.
E83.59 – Other disorders of calcium metabolism
237900002 – Calciphylaxis
- Cholesterol emboli
- Cellulitis – No association with chronic renal failure and does not have the presence of tissue necrosis and ulceration.
- Antiphospholipid syndrome
- Coumadin necrosis – Indurated, necrotic areas on the breasts, thighs, and buttocks.
- Disseminated intravascular coagulation
- Nephrogenic systemic fibrosis
- Lupus profundus lesions may have calcification on x-ray.
- Dermatomyositis and CREST syndrome may have associated calcification.
- Myxoma emboli (atrial myxoma)
- Pancreatic panniculitis
- Ischemia peripheral vascular disease (ischemic ulcer)
- Pyoderma gangrenosum
- Granulomatosis with polyangiitis (formerly Wegener granulomatosis)
- Cocaine levamisole toxicity
- Hyperoxaluria – Oxalate deposition may cause similar deposition in vessels and is also associated with renal failure.
- Atherosclerotic vascular disease
- Stasis ulcer
- Calcinosis cutis
- Livedoid vasculopathy
- Purpura fulminans
- Martorell ulcer
Last Updated: 02/13/2019