Multidisciplinary management of calciphylaxis: a series of 5 patients at a single facility

Abstract

Calciphylaxis is a rare and severe disease that manifests with painful skin ulceration and necrosis. Herein, we report five patients of hemodialysis patients with skin biopsy-proven calciphylaxis at a single facility. One patient had undergone parathyroidectomy (PTx) due to severe secondary hyperparathyroidism, four had been treated with vitamin D receptor activators, and two were on warfarin therapy. All patients had hyperphosphatemia, and one had hypercalcemia. The intact parathyroid hormone level at diagnosis was 2 pg/ml in the patient after PTx, while three patients were within the target range. The average period after diagnosis of calciphylaxis was 2 months. Skin lesions were present on the thighs and lower legs in two patients, and on the dorsum of the foot in one patient. In skin biopsy, calcification was found in the arteriolar media in four patients, and calcium (Ca) was deposited in the dermal lesion in one patient. All patients received local cures, surgical debridement, antibiotics to control infectious diseases, and strict control of serum Ca and phosphate. Calcimimetics were used in all patients except one who had undergone PTx one month before, sodium thiosulfate was used in 4 patients, and low Ca dialysate was used in three patients. The average follow-up period was 7.4 months. Four patients were cured, and one died due to infection. We suggest that multidisciplinary management for infectious diseases, surgical debridement, strict control of mineral and bone markers from the early stage, and elimination of risk factors may improve the course of calciphylaxis, which is a life-threatening disease.

Penile calciphylaxis diagnosis and treatment challenges a case report

“…In most of these cases, it is associated with systemic calciphylaxis [ 8 ]. Calciphylaxis is a rare and severe disease that manifests with painful skin ulceration and necrosis [ 9 ]. This life-threatening condition manifests in very painful nonhealing ischemic lesions due to underlying occlusion of micro vessels in the dermis, subcutaneous, and adipose tissue [ 1 , 2 ].…”

“…Many laboratory and radiological measurements failed to give a solid background for the diagnosis [ 1 , 8 , 9 ]. Penile Doppler ultrasound can be performed to assess the penile vascularity to exclude gangrene.…”

“…CT is sensitive to check for calcifications, and MRI to assess for spreading of ischemia [ 11 ]. Thus, until the time being published research about penile calciphylaxis rely mainly on clinical diagnosis [ 6 , 7 , 9 ]. Clinical diagnosis should be considered in an ESRD patient presented with agonizing pain and erythematous tender penile lesion which might be covered with black eschar [ 7 , 9 ].…”

“…Thus, until the time being published research about penile calciphylaxis rely mainly on clinical diagnosis [ 6 , 7 , 9 ]. Clinical diagnosis should be considered in an ESRD patient presented with agonizing pain and erythematous tender penile lesion which might be covered with black eschar [ 7 , 9 ]. Early diagnosis is essential to prevent delay or improper treatment as it might be aggravated by the introduction of corticoids if misdiagnosed as balanitis [ 6 ].…”

“…Early diagnosis is essential to prevent delay or improper treatment as it might be aggravated by the introduction of corticoids if misdiagnosed as balanitis [ 6 ]. Although biopsy of the lesion is the gold standard for definitive diagnosis [ 8 ], it carries the risk of sepsis and poor wound healing [ 4 , 8 , 9 ]. Hence, there are controversies about performing a biopsy in the standard workup and it is reserved for equivocal cases [ 9 ].…”