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Case report
Acral vascular syndrome during an immune checkpoint inhibitor
  1. Patrick O'Connor1,
  2. Raquele Laury2,
  3. Pooja Bhadbhade3,
  4. Qamar Khan4 and
  5. Stephen Williamson4
  1. 1University of Kansas School of Medicine, Kansas City, Kansas, USA
  2. 2Department of Pathology & Laboratory Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA
  3. 3Department of Allergy, Clinical Immunology, and Rheumatology, University of Kansas Medical Center, Kansas City, Kansas, USA
  4. 4Department of Medical Oncology, University of Kansas Medical Center, Kansas City, Kansas, USA
  1. Correspondence to Patrick O'Connor; poconnor2{at}


Immune checkpoint inhibitors, including antiprogrammed death cell protein 1 (anti-PD-1) and anti cytotoxic T-lymphocyte-associated protein 4 (anti-CTLA-4), have been associated with a range of autoimmune-related side effects since their introduction in cancer treatment. Small vessel digital necrosis, referred to as the acral vascular syndrome, is a rare but serious complication that can result in loss of digits. Here we present a case report of acral vascular syndrome and review possible aetiologies. A 45- year-old woman with invasive ductal carcinoma of the left breast presented to the emergency department during neoadjuvant treatment with carboplatin, docetaxel and pembrolizumab with complaints of severe pain in her right third digit. She had physical findings consistent with ischaemic necrosis and gangrene of the distal phalanx. Angiography demonstrated Raynaud’s phenomenon in the distal portion of the digits. Laboratory testing showed a weakly positive RNA polymerase III antibody level. Her case resulted in surgical amputation of her affected digit after partial resolution of symptoms with prednisone, vasodilators and antibiotics.

  • oncology
  • unwanted effects / adverse reactions
  • vasculitis

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  • Contributors PO is the primary author and he drafted most of the manuscript. PB contributed substantially to the design and drafting of the manuscript, particularly in the description of the disease process. QK contributed to drafting of the manuscript. SW oversaw the drafting process and was responsible for final editing.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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