CASE SERIES Published online at https://www.acpjournals.org/ doi/10.7326/aimcc.2022.0441 Open Access This is an open access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND), which allows reusers to copy and distribute the material in any medium or format in unadapted form only, for noncommercial purposes only, and only so long as attribution is given to the creator. See: https://creativecommons. org/licenses/by-nc-nd/4.0/legalcode. Publication date: 1 November 2022 Disclosures Disclosure forms are available with the article online. Corresponding Author Thomas Hilton, MD, MAcc; Wake Forest School of Medicine, 475 Vine St, Winston Salem, NC 27101; e-mail, [email protected]. How to Cite Hilton T, Bowers N, Strowd L, et al. Point-of-care ultrasound: an emerging tool for diagnosing calciphylaxis. AIM Clinical Cases. 2022;1:e220441. doi:10.7326/aimcc.2022.0441 Point-of-Care Ultrasound: An Emerging Tool for Diagnosing Calciphylaxis Thomas Hilton, MD, MAcc 1 ; Nathan Bowers, MD, PhD 1,2 ; Lindsay Strowd, MD 1,2 ; and Christopher T. Kelly, MD 1,3 1 Wake Forest School of Medicine, Winston Salem, North Carolina 2 Department of Dermatology, Atrium Health Wake Forest Baptist, Winston Salem, North Carolina 3 Department of Internal Medicine, Atrium Health Wake Forest Baptist, Winston Salem, North Carolina Keywords POCUS, Ultrasound imaging, Biopsy, Soft tissues, Medical risk factors, Case series, Necrosis, Calciphylaxis Abstract Calciphylaxis is a disease involving medial calcification of dermal arterioles, resulting in cutaneous necrosis. Diagnosis is typically clinical, but it is frequently misdiagnosed. Skin biopsy, the gold standard diagnostic test, is hindered by risk, patient refusal, and difficulties with histologic evaluation. This case series investigates using soft tissue point-of-care-ultrasound (POCUS) to aid in diagnosis. It describes ultrasound images obtained from patients diagnosed with calciphylaxis. These potentially pathognomonic sonographic findings in the proper clinical context include superficial hyperechoic foci lining small-caliber vessels with posterior acoustic shadowing. This demonstrates the diagnostic potential of POCUS in evaluating patients with possible calciphylaxis. Background Calciphylaxis or calcific uremic arteriolopathy (CUA) is a disease of vascular calcification with resulting microvessel occlusion and ischemic skin lesions that is frequently misdiagnosed and carries a high mortality risk (1, 2). CUA risk factors include end-stage renal disease, chronic kidney disease (CKD), dialysis dependence, obesity, warfarin use, diabetes mellitus, and hyper- phosphatemia (1). The classic clinical presentation begins as tender, firm subcutaneous nodules progressing to painful retiform purpura. Subsequently, skin and soft tissue necrosis result in ulcers covered by a thick black eschar (3). CUA can clinically mimic other cutaneous disorders, including panniculitis, venous or arterial ulcers, pyoderma gangrenosum, cryoglobulinemia, and warfarin necrosis (4). Patients with CUA frequently require inpatient management due to pain and rapid disease progression. Traditionally, CUA has been diagnosed clinically, but skin biopsy is often used as a confir- matory test with clinical signs and symptoms (5, 6). The current gold standard diagnostic test is a skin biopsy with histologic confirmation of calcium lining arterioles in the deep dermis/subcutaneous fat (6). Histopathologic diagnosis of calciphylaxis has several significant limitations, including a lack of standardized comprehensive histopathologic diagnostic criteria, sampling error, calcium washout on processing, and subtlety of calcium on hematoxylin−eosin staining. These limitations impact the sensitivity of skin biopsy as a diagnostic tool (2, 7–9). Histologic diagnosis can be improved using a calcium-specific stain like von Kossa, but this is not always available. The punch biopsy procedure is painful and poses some risk for precipitating ulceration, bleeding, infection, and tissue necrosis. Different imaging modalities for diagnosing CUA, including mammography, plain radio- graphy, and bone scintigraphy, have been studied but not widely implemented. Barriers to these diagnostic techniques include pain associated with mammography, plain film’s inadequate sensitivity to capture small-caliber vessels, the lack of access to nuclear medicine, and bone scintigraphy costs (10–12). In addition, none of these imaging tech- niques have been studied in large enough patient populations to assess their reliability. 1/5 © 2022 Authors. Published in partnership by the American College of Physicians and American Heart Association Downloaded from https://annals.org by 171.243.67.90 on 05/30/2023.
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