Calciphylaxis is a rare, progressive disorder characterized by subcutaneous adipose and dermal microvascular calcifications, microthrombi, and endothelial damage. It mainly affects patients with chronic kidney disease (CKD), which is also known as calcific uremic arteriolopathy. Skin biopsy is the gold standard for diagnosis, but it is an invasive procedure. Calciphylaxis frequently results in ischemic and nonhealing ulcerations with a high mortality rate. A multidisciplinary targeted approach is the primary treatment method. Vascular calcification, which is a common complication in patients with CKD, cannot completely explain the rapid progression of calciphylaxis. This article reviews the advances in the epidemiological characteristics, risk factors, and diagnosis, including non-uremic calciphylaxis and visceral calciphylaxis, pathogenesis, associated animal models, and treatment of calciphylaxis. The scarcity of animal models that mimic the clinical presentation of calciphylaxis hampers the understanding of its pathogenesis. The acute effects on progressive vascular injury, including the induction of severe ischemia and inflammatory responses, have been emphasized. Actively listening to the voices of patients and their families and building a multidimensional research system with artificial intelligence technologies based on the specific molecular makeup of calciphylaxis patients will help tailor regenerative treatment strategies. Mesenchymal stem cells (MSCs) may represent a novel therapy for calciphylaxis because of their regenerative effects, inhibition of vascular calcification, anti-infection and immunomodulation properties, and improvement of hypercoagulability. Safe, effective, accessible, and economical MSC strategies guided by biomarkers deserve consideration for the treatment of this devastating disease.

Calciphylaxis is a rare, life-threatening condition in end-stage renal disease, often presenting with skin ulcers and potentially involving the internal organs. A 62-year-old man undergoing hemodialysis developed non-healing leg ulcers that were initially treated for cellulitis. The diagnosis was confirmed through a biopsy after ulceration and necrosis progressed. Despite multidisciplinary treatment, the patient died because of sepsis and intestinal perforation. Autopsy revealed extensive visceral calciphylaxis, highlighting the under-recognized systemic nature of the disease. This case emphasizes the need for heightened awareness and an early diagnosis as well as the role of autopsies in understanding calciphylaxis, particularly its potential to involve visceral organs, which may be underrecognized.

Calciphylaxis, or calcific uremic arteriolopathy, is a rare condition with a poorly understood pathophysiology. It is often associated with diseases that cause abnormalities in calcium metabolism, such as end-stage renal disease (ESRD) and hyperparathyroidism. While it most often affects the skin, involvement of extracutaneous organs, called systemic calciphylaxis, has been reported.Interestingly, other risk factors have now been identified with the development of calciphylaxis including recent infections, corticosteroid or warfarin use as well as diabetes mellitus, autoimmune diseases, and protein C or S deficiency.We present a 48-year-old female patient with a history of human immunodeficiency virus infection, and recent hospitalizations for acute pancreatitis and COVID-19 who presents with nonspecific abdominal pain, weakness, and mild erythematous skin lesions. Subsequently, the patient developed acute vision loss, severe hypertension, and acute kidney injury with significantly worsening skin lesions. Ultimately, the patient deteriorated rapidly and succumbed. At autopsy, cutaneous calciphylaxis was confirmed, affecting over 50% of body surface area as well as widespread organ involvement by visceral calciphylaxis, most strikingly in the heart and lungs. This case highlights the importance of considering COVID-19 and other nonuremic risk factors as being a trigger for developing catastrophic systemic calciphylaxis.

Background: Calciphylaxis is associated with end-stage renal failure or kidney transplant, although, cases of non-uremic calciphylaxis have been reported. It is a rare disorder, with high mortality, characterized by vascular calcification within cutaneous vessels; vascular calcification can occur in other organs, besides skin, like heart, lung, or pancreas, which is named visceral calciphylaxis.Objective: The purpose was to review current knowledge regarding diagnosis and therapeutic approach to cutaneous calciphylaxis.Methods: A literature review has been conducted associated to word "calciphylaxis".Results: The diagnosis is based mainly on clinical features and histologic findings. Therapeutic options are still controversial.Conclusions: Cutaneous calciphylaxis is a puzzling disease, with a challenging diagnosis, and a complex treatment, which requires a multidisciplinary team and expertise.

A 19-year-old woman, with normal kidney function, was diagnosed as having visceral calciphylaxis, especially diffuse breast and lung calcification. The calcification findings were clearly shown on CT, and bone scintigraphy revealed diffuse uptake in the thoracic area. Although there was no standard therapeutic approach for this clinical setting, the patient received empiric therapy with sodium thiosulfate. After 5 months of consecutive therapy, the calcification condition had a striking regression.