Calciphylaxis
| Calciphylaxis | |
|---|---|
| Other names | Calcific Uremic Arteriolopathy (CUA) |
| Calciphylaxis on the abdomen of a patient with end stage kidney disease. Markings are in cm. | |
| Specialty | Nephrology |
| Symptoms | Painful necrotic skin lesions |
| Complications | Infection, sepsis |
| Risk factors | Female sex, obesity, use of Warfarin, protein C or S deficiency, hypoalbuminemia, diabetes mellitus, use of vitamin D derivatives (calcitriol, systemic steroids) |
| Diagnostic method | Clinical, skin biopsy may aid diagnosis |
| Treatment | Dialysis, analgesics, surgical wound debridement, parathyroidectomy |
| Medication | Sodium thiosulfate |
| Prognosis | 1-year mortality rate is 45–80% |
| Frequency | 1-4% of all dialysis patients in the U.S. |
Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare syndrome characterized by painful skin lesions. The pathogenesis of calciphylaxis is unclear but believed to involve calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and eventual death of skin cells due to lack of blood flow. It is seen mostly in people with end-stage kidney disease but can occur in the earlier stages of chronic kidney disease and rarely in people with normally functioning kidneys. Calciphylaxis is a rare but serious disease, believed to affect 1-4% of all dialysis patients. It results in chronic non-healing wounds and indicates poor prognosis, with typical life expectancy of less than one year.
Calciphylaxis is one type of extraskeletal calcification. Similar extraskeletal calcifications are observed in some people with high levels of calcium in the blood, including people with milk-alkali syndrome, sarcoidosis, primary hyperparathyroidism, and hypervitaminosis D. In rare cases, certain medications such as warfarin can also result in calciphylaxis.