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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. [ABSTRACT FROM AUTHOR]

The article offers information on calciphylaxis which is a complex disorder of microvascular calcification that is typically manifested as painful cutaneous lesions and results in poor outcomes. It mentions that Calciphylaxis occurs in patients with earlier stages of chronic kidney disease, acute kidney injury, and kidney transplantation. It states that extracutaneous vascular calcifications leads to skeletal myopathy, intestinal bleeding and visual impairment.

Calciphylaxis, a disorder of dermal arteriolar calcification with a distinct predilection for patients with advanced kidney disease, remains an enigmatic condition that challenges clinicians. Observations regarding positive associations between levels of circulating parathyroid hormone (PTH) and the risk of calciphylaxis, combined with experimental data suggesting a pathogenic role of secondary hyperparathyroidism in vascular calcification, have led to the assumption that reducing PTH levels will improve the outcomes in patients with calciphylaxis. This editorial communicates the contemporary data regarding surgical parathyroidectomy as a treatment option for these patients. We raise concerns regarding the quality of available data and discuss the direction of future comparative effectiveness research in this field. [ABSTRACT FROM AUTHOR]