Calciphylaxis and Systemic Calcinosis

Severe Calciphylaxis in a Renal Patient on Long-Term Oral Anticoagulant Therapy

“…However, the majority of patients with calciphylaxis suffer from end-stage renal disease and secondary hyperparathyroidism, treated eith er by chronic dialysis or renal transplantation [8,9,11,15,16]. Thus, several factors such as azotemia, high levels of PTH, calcium and phosphorus, a high calcium-phosphate product, and vitamin D treatment are believed to interact in the pathogenesis of this condition.…”

“…Calciphylaxis has been observed in patients with renal allografts whose serum creatinine levels were normal or close to normal and in patients with primary hyperpara thyroidism without underlying renal disease [8,11,19].…”

“…It must be distinguished from calciphylaxis, a rare calcifica tion syndrome of peripheral ischemic necrosis, first de scribed by Bryant and White [6], Livedo reticularis is often the first clinical manifestation in calciphylaxis pro gressing rapidly to painful ischemic necrosis and ulcer ation of fingers, arms, buttocks, legs and toes. Not only arteries can be affected, but also visceral, periarticular, cutaneous and corneal sites [7,8], Death is usually due to sepsis [9][10][11]. Histologically, small and medium-sized arteries display intense medial calcification with intimai thickening, and diffuse calcifications are found in sur rounding soft tissues [8,9,12], Although the pathogenic mechanisms resulting in cal ciphylaxis are not known precisely, various factors such as azotemia, increased parathyroid hormone (PTH) levels, a high calcium-phosphate product, or excess vitamin D are believed to be involved.…”

Chronic Renal Failure and Skin Calcifications

“…Parathyroidectomy is only mean ingful in an early stage [11 ]. In the case of the extensive gangrene, surgical debridement is necessary to prevent sep sis.…”

“…The pathogenesis of these calcifications with chronic renal failure is not yet completely understood [11]: apart from the classic Ca-P disturbances due to poor renal func tion, all authors mention the theory of Selye concerning the concept of 'calciphylaxis' [12] for lack of a better explana tion. In this process, defined by Selye in 1962 in an experi mental model with rats, two important phases are distin guished |7, 10]: (1) first and foremost, the sensitization: the tissues arc sensitized for calcification by the administration of a systemic calcifying factor; such calcifying factors can be parathormone, vitamin D, hypercalcemia or even the induction of renal failure; (2) then follows the precipitation by the administration of a 'challenging agent' after a critical period; these challengers are very aspecific and can be an albumin infusion, corticosteroids, immunosuppressiva or even a minor trauma.…”