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.
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http://dx.doi.org/10.1097/PAF.0000000000001035 | DOI Listing |
Am J Forensic Med Pathol
March 2025
From the Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY.
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.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, California Institute of Behavioral Neurosciences and Psychology, Fairfield, USA.
Calciphylaxis is a rare but potentially life-threatening disease that is not yet completely understood. It occurs mainly in patients with chronic kidney disease termed calcific uremic arteriolopathy (CUA) but also affects patients with normal renal function. Although this disease's pathogenesis is unclear, it is associated with the dysregulation of calcium and phosphate and subsequent calcification of peripheral arterioles.
View Article and Find Full Text PDFAm J Dermatopathol
December 2024
Pathologist, Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY.
Background: Microvascular thrombosis is key to the pathogenesis of calciphylaxis. C5b-9-mediated microvascular injury reflective of complement pathway activation could be a key pathophysiologic event.
Methods: We conducted a retrospective multicenter study of 24 patients who have had biopsy-supported calciphylaxis from the 2010-2022 data base from Emory where C5b-9 immunohistochemistry (IHC) had not been conducted and the 2019-2023 data base from Cornell where C5b-9 IHC was done as part of the routine calciphylaxis work up.
Background: Calciphylaxis is a rare and serious complication in patients with kidney disease. It has few treatment options and poor prognosis. Hyperbaric oxygen therapy (HBOT) may improve wound healing and was added to our conventional care in 2012.
View Article and Find Full Text PDFWounds
October 2024
UMass Chan Medical School, Department of Dermatology, Worcester, MA.
Background: Calciphylaxis is a rare and life-threatening condition characterized by cutaneous necrosis resulting from vessel calcification and thrombosis. Commonly associated with end-stage renal disease and hyperparathyroidism, calciphylaxis presents as retiform purpura evolving into necrotic eschars.
Case Report: This report details an atypical case of non-nephrogenic unilateral bullous calciphylaxis in a 71-year-old female, emphasizing the importance of considering calciphylaxis in the differential diagnosis of bullous disorders.
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