Calciphylaxis is a rare, often fatal condition, characterized by progressive cutaneous necrosis that frequently occurs in patients with end-stage renal disease. It is seen almost exclusively in patients with end stage renal disease. It results in chronic non-healing wounds and requires parathyroidectomy and hyperbaric therapy. Calciphylaxis is a rare but serious disease. Calciphylaxis typically develops after the start of dialysis and is seen in approximately 1% of patients with chronic renal failure. Calciphylaxis is characterised by systemic medial calcification of the arteries, ie calcification of tunica media.
Unlike other forms of vascular calcifications calciphylaxis is characterised also by small vessel mural calcification with or without endovascular fibrosis, extravascular calcification and vascular thrombosis, leading to tissue ischaemia. Calciphylaxis is an uncommon condition that affects 1-4% of the population with ESRD. Calciphylaxis most commonly occurs in patients with end-stage renal disease who are on haemodialysis. It also has been reported in patients with breast cancer (treated with chemotherapy), liver cirrhosis , cholangiocarcinoma, Crohn’s disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE).
Calciphylaxis can occur in those with high or normal levels of serum calcium and phosphate, with or without vitamin D replacement, in dialysed patients and less often in those who have not yet started dialysis or in those who have received a renal transplant. It is more common in women than in men, in obese patients compared to those of normal weight. Small blood vessels become blocked with blood clots, which leads to the black painful necrotic areas. It is thought that the clots occur because of calfication within the walls of the blood vessels. The best treatment for calciphylaxis is not yet clear.
Medical care is mainly supportive. Parenteral iron therapy, calcium supplementation, and vitamin D supplementation also helpful this condition. Rigorous and continuous control of phosphate and calcium balance most probably will avoid the metabolic changes which may lead to calciphylaxis. In patients with significantly elevated parathyroid hormone that cannot be medically controlled, surgical removal of the parathyroid glands has been shown to reduce pain and promote wound healing. Use of a vacuum-assisted closure device has been successful in several cases of calciphylaxis after extensive debridement and prior to skin grafting.