Calciphylaxis
Calciphylaxis Causes
The development of calciphylaxis are associated with certain disorders such as chronic renal failure, hypercalcemia, hyperphosphatemia, elevated calcium-phosphate product, hyperparathyroidism, and vascular calcification. Some speculative associations include aluminum toxicity, coagulation abnormalities, and iron dextran infusion. The cause of calciphylaxis however remains unclear. Though most cases occur in the setting of chronic renal failure, abnormal calcium-phosphate homeostasis, and hyperparathyroidism, the disease may still occur in normal settings or minimally elevated calcium and phosphate levels.
Calciphylaxis Definition
Calciphylaxis is a highly morbid syndrome of vascular calcification and skin necrosis that is poorly understood. Mostly seen exclusively in patients with end stage renal disease, Caciphylaxis results in chronic non-healing wounds and requires parathyroidectomy and hyperbaric therapy. Considered as a rare but serious disease, calciphylaxis is a type of extraskeletal calcification that is similar to those observed among patients with hypercalcaemic states such as those with milk alkali syndrome, hyperparathyroidism, sarcoidosis and hypervitamino sis D.
Calciphylaxis Diagnosis
Though there is a clinical diagnosis for Calciphylaxis, there is no diagnostic test for the disease. The known characteristics are ischaemic skin lesions and the appearance of necrotic skin lesions. These are considered suspected diagnosis and can be confirmed by conducting a skin biopsy. The bone scintigraphy may show an increased tracer accumulation in the soft tissues as well as an arterial calcification and occlusion in the absence of vasculitis. There is also no specific treatment for Calciphylaxis. There are acceptable treatments however but none is internationally recognized as standard care. Among those, include dialysis, intensive wound care, adequate pain control, urgent parathyroidectomy and correction of the underlying plasma calcium and phosphorus abnormalities.
Calciphylaxis Historical Background
It is observed that patients with a long-standing history of chronic renal failure and renal replacement therapy are most likely to have calciphylaxis. It may even occur before the replacement therapy is initiated on rare cases. Also, many of those who develop the disease went through renal allograft transplantation. Typically, Calciphylaxis lesions develop suddenly and progress rapidly. The lesions may also be single or numerous and generally occur in the lower extremities. Certain factors may also trigger the development of Calciphylaxis among patients. Said factors include, long-term obesity, malnutrition, recent and sudden weight loss, liver disease, diabetes mellitus among others. A review of the patient's medical record more often than not reveals a history of hyperphosphatemia with hyperparathyroidism and hypoalbuminemia.
Calciphylaxis Symptoms and Signs
Calciphylaxis is characterised by systemic medical calcification of the arteries such as the calcification of tunica medica. It is also characterized by a small vessel mural calcification with or without endovascular fibrosis, extravascular calcification and vascular thrombosis that may lead to tissue ischaemia.