Calciphylaxis
Definition: Patients with end-stage renal disease (ESRD) are disproportionately affected by calciphylaxis, also known as calcific uremic arteriolopathy, an uncommon and frequently fatal syndrome marked by vascular calcification and skin necrosis(1).
Etiology: Dysregulated metabolism of calcium phosphate causes calcification of small blood arteries, which in turn causes tissue ischemia and necrosis in cases of calciphylaxis (1). Hyperparathyroidism, liver disease, end-stage renal disease (ESRD), and prior use of corticosteroid or vitamin K antagonists are risk factors(1).
Epidemiology:
Cases have been reported worldwide with an estimated mortality rate ranging from 40-80%(1).
Signs: Patients experience painful, retiform purpuric skin lesions that eventually become ulcerations and necrosis(1).
Symptoms: Patients may report severe discomfort, frequently out of proportion to the skin findings, at the location of necrotic lesions(1) Sepsis, fever, and indications of organ ischemia are examples of systemic symptoms.
Differentials: Necrotising fasciitis, cryoglobulinemia, anti phospholipid syndrome, coumarin necrosis and vasculitis (2).
Diagnosis: A deep wedge skin biopsy demonstrating extravascular and vascular calcium deposits in subcutaneous and dermis layers, fibrin thrombi, ischemic necrosis of the epidermis and fat necrosis supports the clinical diagnosis (1,3). Vascular calcifications may be shown on imaging, and hyperphosphatemia or hypercalcemia may be seen in laboratory tests (2).
Treatment: The management entails wound care, pain management, and reducing calcium and phosphate levels to address the underlying mineral imbalance (2). Commonly used treatments include bisphosphonates and sodium thiosulfate (2). Necrotic tissue may require surgical debridement, although the prognosis is still not good (2).
References:
- Rick J, Strowd L, Pasieka HB, et al. Calciphylaxis: Part I. diagnosis and pathology. Journal of the American Academy of Dermatology. 2022;86(5):973-982. doi:10.1016/j.jaad.2021.10.064
- Calciphylaxis: Causes, symptoms, and management – dermnet. DermNet®. September 23, 2024. Accessed September 26, 2024. https://dermnetnz.org/topics/calciphylaxis.
- Gupta S, Baby D, Upadhyay M, et al. Calciphylaxis and its diagnosis: A Review. Journal of Family Medicine and Primary Care. 2019;8(9):2763. doi:10.4103/jfmpc.jfmpc_588_19