A Rare Case of Multiorgan Calciphylaxis in a Patient with Stage 5 Chronic Kidney Disease

Abstract

Calciphylaxis or calcific uremic arteriolopathy (CUA) is a potentially life-threatening vasculopathy involving the skin and subcutaneous tissues. It is usually associated with chronic kidney disease (CKD) and rarely with acute renal failure or predialysis patients. The clinical diagnosis of calcific uremic arteriolopathy relies on high index of suspicion. CUA is commonly associated with secondary hyperparathyroidism and high serum calcium and phosphate products. Moreover, using biopsy as a diagnostic tool is controversial, due to the high risk of poor wound healing and sepsis. Radiological studies usually reveal extensive calcification of branching vessels such as penile arteries, eventually leading to gangrene formation in extremities and penis. Histopathological analysis confirms the diagnosis of calcific uremic arteriolopathy and rules out the presence of malignancy. CUA is a systematic disease that involves multiple organs, and to the best of our knowledge this is the first reported case involving the penis, bladder, and eyes.

Penile necrosis by calciphylaxis leading to gangrene in a patient with chronic renal failure on dialysis: A case report

“…In previously reported cases [ [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] ] ( Table 1 ), all patients including the present case had chronic kidney disease (78% were reported on dialysis) and diabetes mellitus which could be a predisposing factor [ 16 ]. Seven reported cases, including our patient’s case, had a high blood pressure and two of them had a long history with smoking [ [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] ]. In our case, other risk factors were found such as obesity and anticoagulant treatment that may worsen pre-existing atherosclerosis.…”

“…In our case, the histopathological diagnosis of calciphylaxis shows diabetic microangiopathy that is accompanied by vascular calcification of both the tunica intima and media of blood vessels with intimal fibroblastic proliferation. In other cases [ [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] ], the histopathological diagnosis was performed in 6 patients including our case and 8 patients who received at least one radiological assessment. In our case, the patient received an arteriography that showed multiple vascular calcifications at the level of the internal iliac artery and its branches.…”

Penile gangrene from calciphylaxis is salvageable with intravenous sodium thiosulfate and early total parathyroidectomy

“…Specific to penile calciphylaxis, a literature search revealed only six reported cases that used STS. Four cases were used in combination with surgical interventions including debridement and/or penectomy as the initial form of treatment; three out of the four patients survived at the time of reporting [ [11] , [12] , [13] ] while one out of the four patients was readmitted a week later for urosepsis and demised [ 14 ]. The fifth case was a patient with initial good recovery of penile lesion but subsequently readmitted for wet gangrene of the penis four weeks later due to poor wound care [ 15 ].…”

A rapid unfavorable penile calciphylaxis case followed by total penectomy

“…The etiological factors of calciphylaxis are abnormalities in the calcium‐phosphate products (ie, elevated calcium‐phosphate product levels and secondary hyperparathyroidism). The other risk factors are AVK intake, the presence of diabetes mellitus or obesity, female sex, and Caucasian origin 2,3 . Two forms of calciphylaxis are described: a proximal form that affects the trunk and the limbs above the elbows and knees and a distal form that evolves the distal ends of the limbs.…”