Volume 46, Issue 1 pp. 16-25
ORIGINAL ARTICLE

Factors associated with false-negative pathologic diagnosis of calciphylaxis

Erik A. Williams

Corresponding Author

Erik A. Williams

Massachusetts General Hospital, Dermatopathology Unit, Department of Pathology, Boston, Massachusetts

Harvard Medical School, Boston, Massachusetts

Correspondence

Erik A. Williams, MD, Massachusetts General Hospital, 55 Fruit Street, WRN-245, Boston, MA 02114.

Email: [email protected]

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Andrea P. Moy

Andrea P. Moy

Department of Dermatology, Northwell Health and Zucker School of Medicine at Hofstra/Northwell, Lake Success, New York

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Nicole A. Cipriani

Nicole A. Cipriani

The University of Chicago Medicine & Biological Sciences, Chicago, Illinois

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Sagar U. Nigwekar

Sagar U. Nigwekar

Harvard Medical School, Boston, Massachusetts

Massachusetts General Hospital, Division of Nephrology, Department of Medicine, Boston, Massachusetts

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Rosalynn M. Nazarian

Rosalynn M. Nazarian

Massachusetts General Hospital, Dermatopathology Unit, Department of Pathology, Boston, Massachusetts

Harvard Medical School, Boston, Massachusetts

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First published: 02 October 2018
Citations: 23

Abstract

Background

Calciphylaxis is a rare, painful, and debilitating disorder of vascular calcification and skin necrosis that typically affects patients with advanced kidney disease. During our routine pathology practice, we noted several missed diagnoses on calciphylaxis consultation cases originating from outside institutions and sought to explore factors associated with false-negative pathologic diagnosis of calciphylaxis.

Methods

The pathology database of a large tertiary academic medical center was retrospectively searched for “calciphylaxis” in inside reports on outside surgical consultation cases between 2007 and 2017. Inside and outside pathology reports were compared and medical records were searched for calciphylaxis clinical diagnosis and risk factors.

Results

Twenty-four calciphylaxis patients were identified, with median age of 63.5 years. Seven of 24 (29%) of specimens were inadequate (e.g., lack of subcutaneous adipose tissue for evaluation). Eight of 17 (47%) of adequate specimens had a first false-negative pathologic diagnosis of calciphylaxis. Histochemical staining for calcium significantly correlated with true-positive diagnosis (93% vs 55%, P = 0.004). Dermatopathology fellowship training significantly correlated with true-positive diagnosis (82% vs 38%, P = 0.047).

Conclusions

Adequate sampling, dermatopathology training, and use of histochemical stains to identify calcium associate with decreased false-negative rate for calciphylaxis diagnosis. These findings need further evaluation in larger prospective studies.

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