Calciphylaxis With Normal Renal and Parathyroid Function

Abstract

Background: Calciphylaxis is a life-threatening form of metastatic calcification-induced microvascular occlusion syndrome. Although traditionally observed in patients with end-stage renal disease and/or hyperparathyroidism, the development of calciphylaxis in "nontraditional" patients having both normal renal and parathyroid function has been reported. However, to date there has been no collective analysis identifying common patient characteristics potentially predisposing to the development of calciphylaxis in nontraditional patients.Observations: A 58-year-old woman with endometrial carcinoma developed extensive calciphylaxis despite the presence of normal renal and parathyroid function. The disease resolved with rapid diagnosis, supportive therapy, and medical management. Analysis of this case and the 13 previously reported cases of nontraditional calciphylaxis identified the following patient characteristics that highlight clinical situations potentially predisposing to calciphylaxis: hypoalbuminemia, malignant neoplasm, systemic corticosteroid use, anticoagulation with warfarin sodium or phenprocoumon, chemotherapy, systemic inflammation, hepatic cirrhosis, protein C or S deficiency, obesity, rapid weight loss, and infection.Conclusions: Calciphylaxis is becoming increasingly common in patients with normal renal and parathyroid function. The observations from this study may assist dermatologists in the rapid diagnosis and prompt initiation of therapy for this devastating disease.

Calciphylaxis and Martorell Hypertensive Ischemic Leg Ulcer: Same Pattern - One Pathophysiology

“…'Calciphylaxis in normal renal function' [17] consistently affects morbidly obese people suffering from arterial hypertension and type 2 diabetes as a result of their metabolic syndrome [7] . The disease is characterized by the rapid appearance of progressive skin infarctions in the regions of the inner thigh, fatty abdominal apron, breasts or outer upper arms -i.e.…”

“…Ramsey-Stewart [18] described the entity as 'eutrophication', and Kalajian et al [17] reported on a series of patients under the more descriptive term 'calciphylaxis with normal renal and parathyroid function'. Hackett et al [19] reported successful treatment with sodium thiosulfate, shortly after the introduction of this modality, for the treatment of severe cases of classical calciphylaxis.…”

“…Calciphylaxis with normal renal and parathyroid function (synonym: eutrophication) cannot be clinically and histopathologically distinguished from the proximal pattern of classical calciphylaxis -with the difference that these patients have normal kidney function [17] . The skin of the inner thighs, abdominal fatty apron, breasts and the outer upper arms develops severe livedo which can rapidly progress to become extremely painful skin infarctions ( fig.…”

Calciphylaxis: a severe complication of renal disease

“…It occurs in up to 4% of patients undergoing long-term dialysis, but sporadic instances in patients with normal renal function have also been reported. 1,2 In addition to kidney failure, other risk factors include obesity, female sex, white race, liver disease, use of systemic corticosteroids, decreased albumin levels and elevated calcium-phosphorus products. 2,3 Parathyroid hormone is often found to be abnormal.…”

“…1,2 In addition to kidney failure, other risk factors include obesity, female sex, white race, liver disease, use of systemic corticosteroids, decreased albumin levels and elevated calcium-phosphorus products. 2,3 Parathyroid hormone is often found to be abnormal. Mortality associated with calciphylaxis is high, with an estimated one-year rate of survival of 45%.…”