Calciphylaxis: Diagnostic and Treatment Advances for the Inpatient Dermatologist
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HOSPITAL-BASED DERMATOLOGY (L GUGGINA AND C NGUYEN, SECTION EDITORS)
Calciphylaxis: Diagnostic and Treatment Advances for the Inpatient Dermatologist Gowri Kabbur 1,2 & Daniel D. Miller 1,3 Accepted: 9 October 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Calciphylaxis is a rare, life-threatening condition that frequently requires inpatient dermatology management. This article provides a comprehensive overview of the risk factors, classification, and pathogenesis of calciphylaxis, and summarizes current evidence for diagnostic testing and multi-disciplinary therapeutic management. Recent Findings The two major subtypes of calciphylaxis tend to present in different anatomic locations, with non-uremic lesions favoring the distal extremities. Skin biopsies obtained on the distal extremities are more sensitive in detecting calciphylaxis-related vascular calcifications, compared to other sites. Underlying hypercoagulable disorders are common among calciphylaxis patients, implicating thrombosis as a major mediator of this disease and highlighting the potential therapeutic role of anticoagulants. Summary Calciphylaxis is a challenging condition to treat, requiring multi-disciplinary care from dermatology, nephrology, pain medicine, plastic/general surgery, and palliative care. Inpatient dermatologists should be aware of the risk factors associated with calciphylaxis and focus therapy with pharmacologic agents and local wound care. Keywords Calciphylaxis . Calcific uremic arteriolopathy . Retiform purpura . Hypercoagulable disorders . Vascular calcifications . Wound care
Introduction Calciphylaxis is a rare disorder of subcutaneous vascular calcification and thrombosis that typically presents in skin as painful, necrotic retiform purpura and ulcers. Calciphylaxis is classically associated with chronic kidney disease (CKD), specifically end-stage renal disease (ESRD) patients on dialysis; however, multiple cases have been reported in the absence of renal disease (non-uremic disease). While large epidemiologic studies are lacking, one retrospective review estimates a This article is part of the Topical Collection on Hospital-Based Dermatology * Daniel D. Miller [email protected] 1
Department of Dermatology, University of Minnesota, Minneapolis, MN, USA
2
Department of Internal Medicine, University of Minnesota, Minneapolis, MN, USA
3
Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, MN, USA
4% prevalence among ESRD patients on hemodialysis; newer reports suggest increasing incidence in this population [1, 2]. Calciphylaxis is a true dermatologic emergency with significant morbidity and mortality, with a reported median survival of 2.6 to 10.8 months from the time of diagnosis [3••, 4, 5]. Reported 1-year mortality rates for calciphylaxis range between 26 and 80%, with sepsis as the leading cause of death [3••, 4–7]. In one cohort study of 63 patients, the 1-year mortality rate for peritoneal dialysis patients with calciphylaxi
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