Cutaneous necrosis by calcific uremic arteriolopathy

Abstract

Background Calcific uremic arteriolopathy is a rare and serious disorder characterized by systemic medial calcification of the arteries and tissue ischemia. Most often it is found in patients with chronic renal failure on dialysis and in renal transplant recipients with secondary hyperparathyroidism. MethodsWe report six patients with end-stage renal disease [five on hemodialysis (one with a nonfunctioning renal graft) and one on peritoneal dialysis] who developed painful livedo reticularis and skin necrosis of the limbs. All had secondary hyperparathyroidism and elevated calcium-phosphorus product. Our patients presented with the following clinical features: white race (six patients), hypoalbuminemia (three patients), diabetes (one patient), and obesity (four patients). ResultsSubtotal parathyroidectomy was performed in three cases. Despite this procedure, two patients died; one patient survived and his lesions healed. One patient was treated with aggressive wound care and hemodialysis (with low dialysate calcium concentration and Renagel phosphorus binders) and one patient received only local wound care, both with improvement of their lesions. In one case, no therapy was performed because the patient died immediately after diagnosis. ConclusionsThe three patients who survived (Cases 4, 5 and 6) had distal lesions, normal serum albumin, and an early diagnosis. There was a relationship between the outcome of the patients and these factors, rather than the type of treatment received.

Warfarin-induced skin necrosis

“…35 Calciphylaxis has a high associated morbidity and can result in fatality in up to 80% of cases. 32,35 Cryoglobulinemia. Cryoglobulins are cold-insoluble B-cellederived immunoglobulins (and/or complement) that aggregate and precipitate at temperatures below 378C in vivo or in vitro.…”

“…[33][34][35] The disorder most often involves the lower extremities. 32 Internal derangements of calcium-phosphate metabolism and of the regulatory factors derived from vascular smooth muscle cells including osteopontin, osteoprotegerin, and matrix alpha-carboxyglutamic acid protein, have been implicated in the pathogenesis of this disorder. 35 Although vascular calcifications occur frequently in patients with hemodialysis, progression to tissue ischemia is rare.…”

“…Elastic fiber degeneration and homogenization has also been reported and can be demonstrated with use of the Verhoeff-Van Gieson stain. 32 Patients, typically female, tobacco users, diabetic, and hyperlipidemic, characteristically present with livedo reticularis, and red-purple discolored plaques progressing to tender, ulcerative, gangrenous skin necrosis. [33][34][35] The disorder most often involves the lower extremities.…”

Breast Lesions with Intractable Pain in End-Stage Renal Disease: Calciphylaxis with Chronic Hypotensive Dermatopathy Related Watershed Breast Lesions

“…14,19-21 Urgent parathyroidectomy might be necessary if there is elevated parathyroid hormone level. 22,23 Sodium thiosulfate reduces metastatic tissue calcification, acts as an antioxidant, and induces endothelial nitric oxide synthesis, which improves blood flow and tissue oxygenation leading to ulcer healing. 24,25 Bisphosphonates, both …”

Possible role of hypercoagulability in calciphylaxis: Review of the literature

“…An earlier review of the literature found 39 cases of calciphylaxis in which protein C levels were reported, and 22 cases with reported levels of protein S. 2 The cases included patients with and without renal disease, although the majority of patients had end-stage renal disease. In this review, 36% had decreased protein C levels, and 50% had decreased levels of protein S. We performed a similar review of the more recent literature and found an additional 22 cases of patients with calciphylaxis and reported protein C levels and 18 with levels of protein S. [2][3][4][5][6][7][8][11][12][13]15,[17][18][19][20][21][22] Combining the two literature reviews shows that 38% had decreased protein C levels and 43% had decreased levels of protein S.…”