Calciphylaxis

1723

Rare disease characterized by sudden onset of painful fat necrosis due to vascular medial calcification and thrombosis of small arterioles supplying the adipose tissue.

Painful, indurated, necrotic nodules, typically appear on areas of the body rich in adipose tissue including the abdominal pannus, breast, buttocks, and thighs.

Lesions can become infected and lead to death.

Calcification of the medial layer of small blood vessels.

Can lead to microangiopathy and infarction of tissues.

Risk factors include elevated phosphorus levels, hypoalbuminemia,

Pathological findings include vascular medial calcification and thrombosis of small arterials supplying the subcutaneous tissue.

Mineral bone abnormalities such as hyperphosphatemia, and hyperparathyroidism and their treatments with calcium based phosphorus binders and vitamin D are frequently implicated in the origin of calciphylaxis in patients with ESRD.

Process associated with time ability and mortality.

Etiology remains unknown.

Risk factors include: end-stage renal disease, chronic hemodialysis, female gender, obesity, and hyperparathyroidism.

Rarely the disease has been described in dialysis-naïve patients and in patients with no renal abnormalities.

When it occurs with normal renal function, it occurs mainly in white women.

Nonnephrogenic calciphylaxis is ia rare disease that follows a multi factorial origin with women, white race, obesity, and use of warfarin being the most common demographic and concomitant factors.

One year mortality of 60-80%.

78% of patients with normal renal function and calciphylaxis were taking vitamin K antagonists.

When it occurs with normal renal function evaluation showed serum calcium, phosphorus, vitamin D, parathormone levels are all within normal limits.

Other associated conditions that are present in patients with normal renal function and calciphylaxis are obesity, liver disease, malignancy, autoimmune disease, his diabetes, and nephrolithiasis.

Affects 1-41/2% patients with ESRD.

Other risk factors include warfarin and steroid use.

Incidence on the rise in patients with ESRD.

Many patients are chronically anticoagulated with warfarin.

Frequently coexist with cardiovascular disorders including atrial fibrillation, venous thromboembolism, or heart valve prosthesis.

Warfarin can predispose patients to vascular calcification by inducing gamma-carboxylation inhibition of matrix Gla protein.

Warfarin may impact calcium deposition in blood vessel walls.

A skin process characterized by arterial calcification, thrombosis and skin infarctions.

Patients present with painful subcutaneous patches of skin on the extremities and pannus, that progress to ulceration.

The skin appears in ischemic and infarcts with tender ulcerations.

Histologically characterized by calcifications ofsmall and medium sized dermal and subcutaneous blood vessels.

Most commonly seen in chronic renal failure patients on dialysis.

May be seen in liver disease.

Prognosis is quite poor with an estimated one year survival of 45.8% and a two-year survival of 20% (Weenig RH et al).

No treatment has been effective on a consistent basis.

Treatment consists of lowering serum phosphorus, calcium and parathyroid hormone levels.

Corticosteroids generally not helpful.

Surgical debridement of ulcerated areas may be beneficial.

Parathyroidectomy effectiveness is a matter of debate (Weenig RH).

Finding suggests surgical wound debridement and subtotal parathyroidectomy in patients with end-stage renal disease with hyperparathyroidism can benefit (McCarthy JT).