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Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare vascular disease often associated with ESRD patients. Even more rare is penile calciphylaxis (<100 reported cases) due to the fact that the rich vascular network of the penis protects against ischemia . Both conditions are life-threatening with a high mortality rate of up to 69% at six months and a mean time to death of 2.5 months. We present a case of penile calciphylaxis observed in our institution that, unlike what is usually reported, had a favorable evolution and prolonged survival.
A 49-year-old man consulted in February 2018 for painful glans penis ulcer (Fig 1). One month earlier he had suffered acute left leg arterial occlusion that had been resolved with thrombectomy. Arteriography showed severe arterial calcifications in iliac, femoral, popliteal, tibial and peroneal territories. He had started renal replacement therapy (HD) in 1995 for chronic glomerulonephritis. In 2002 he had been parathyroidectomized and since 2010 he had been on PD for problems with his vascular access. In 2015 he had presented dry gangrene and spontaneous distal phalanx amputation of second toe of his left foot. His blood anaysis demonstrated severe hyperphosphatemia and PTH of 271 pg/ml.
Biopsy of the glans ulcer confirmed the diagnosis of calciphylaxis (Fig 2). The pain was treated with opioids and the patient was transferred to daily HD. He started treatment with sodium thiosulfate (STS) 25 g IV three times a week with good tolerance. After one month, symptomatic improvement was observed and at three months the lesion was resolved (Fig 3). The following three months he continued with mixed dialytic therapy (3 days per week HD and the remaining days, PD with low-calcium dialysate) plus STS (three weekly doses), as the necrotic right foot lesions remained unresolved (Fig 4). In 2019, an arteriography showed severe ostial stenosis of the right primitive iliac artery, and angioplasty with stent implantation was performed. She continued with a three-weekly HD plan and STS once a week until completing 2 years of treatment (cumulative dose 3750 g) with resolution of the ischemic lesions in the lower limbs.
At the time of this report, 5 years and 8 months after diagnosis, the patient is still alive and active at work. It is possible that the early diagnosis, the multidisciplinary approach and the prolonged treatment with STS (2 years) have been related to his unexpected and favorable evolution (Fig 5-6 Lower limbs and penis images 2023)