Management of Cutaneous Calciphylaxis

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Management of Cutaneous Calciphylaxis Vijay Kodumudi . George M. Jeha . Nicholas Mydlo . Alan D. Kaye

Received: August 20, 2020 / Accepted: September 11, 2020  The Author(s) 2020

ABSTRACT Calciphylaxis is a deadly, painful disease with a 1-year mortality of up to 50%. The disease is commonly associated with patients with endstage kidney disease (ESKD), but it can manifest in non-uremic patients as well. In patients who are undergoing dialysis, the incidence of calciphylaxis can range from 0.04% to 4%. The progressive arterial calcification seen in calciphylaxis can affect multiple body organs, including the skin, brain, lungs, and muscle. In

V. Kodumudi University of Connecticut School of Medicine, Farmington, CT, USA G. M. Jeha Louisiana State University Health Sciences Center School of Medicine, New Orleans, LA, USA N. Mydlo Bentley University, Waltham, MA, USA A. D. Kaye (&) Department of Anesthesiology and Pharmacology, Toxicology, and Neurosciences, Louisiana State University Health Sciences Center Shreveport, Shreveport, LA, USA e-mail: [email protected]

cutaneous calciphylaxis, painful and non-healing nodules, plaques, and ulcers may appear, increasing morbidity for patients. Diagnosis can be difficult, and the condition can clinically appear similar to other dermatological diseases, especially in non-uremic patients. Currently, skin biopsy with histological analysis is the most reliable method to help diagnose the condition. In certain cases, the use of medical imaging may be helpful. Treatment of pain in this condition can be difficult and should be multimodal and include wound care as well as modification of risk factors. Analgesic options include opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), as well as analgesic options that are targeted for specific patients. There are currently multiple clinical trials underway that are studying targeted therapies for this condition.

Keywords: Analgesia; Calcific uremic arteriolopathy; Cutaneous calciphylaxis; End stage renal disease; Pain; Wound management

Adv Ther

Key Summary Points Calciphylaxis is a progressive disease with high mortality. Treatment of pain must be multimodal and should target the underlying disease etiology, as pain is often associated with the primary disease burden. Any attempt at analgesia and symptomatic management should be coupled with proper wound care and modification of underlying risk factors. Various available therapeutic options include parathyroidectomy, sodium thiosulfate, optimization of renal impairment, cessation of offending drugs, and multimodal analgesic regimens. Currently, there are multiple clinical trials in progress in order to provide relief to patients with this debilitating disease.

0.04% to 4%, and studies report that the incidence has been rising over the last decade [3]. Calciphylaxis is a deadly disease, with the mortality at 6 months being 30% and the mortality at 12 months being 50% [4]. In addition to a high mortality, the disease is highly morbid, with many patients experiencing