We recently encountered a rare case, for which we performed a case study in conjunction with a brief literature review. This was accepted for publication in Baylor University Medical Center Proceedings.
Calciphylaxis was first seen in 1898, and later established in 1962 as a disease state involving calcification of multiple organs resulting in tissue death (necrosis). In humans it is most commonly seen in patients with chronic kidney disease on hemodialysis (approximately 1-4% of these patients). It remains poorly understood, and treatments are lacking. Overall, calciphylaxis’ 6 month survival is around 50% – making this a highly morbid disease. Involvement of highly vascularized areas such as the penis, provides an even more grim prognosis (mortality rate up to 69% at 6 months). Calciphylaxis typically presents as exquisitely tender and painful noduloplaques covered in eschar, tissue ischemia, and poor wound healing present difficult clinical challenges.
Two treatment approaches are indicated and are patient dependent: interventional and non-interventional. Interventional solutions can involve parathyroidectomy, excision of the involved area, or revascularization. Non-interventional approaches typically involve medical treatment such as sodium thiosulfate, cinacalcet, bisphosphonates, vitamin K, and wound care.
Primary prevention strategies for high-risk patients involve improvement of nutritional status (both obesity and malnourishment increase risk), vitamin D supplementation, routine monitoring of serum calcium and phosphate, minimizing trauma (such as injections) at high-risk sites (buttocks, thighs, abdomen), and avoiding hypotension. A low-calcium dialysate could also be beneficial.
