Histopathology of Calciphylaxis

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Abstract

Calciphylaxis is a rare, painful, and life-threatening condition with a high mortality rate. Although the etiology of calciphylaxis is not well understood, it has been proposed that calcium deposition within and around subcutaneous vessels restricts blood flow chronically, thereby predisposing the patient to acute pannicular and dermal thrombosis. Given increasing recognition of the role of hypercoagulability in calciphylaxis, this retrospective cohort study sought to evaluate the presence of thromboses and dermal angioplasia in calciphylaxis. Moreover, we aimed to validate previous observations about the histopathology of calciphylaxis compared with skin biopsies from patients with end-stage renal disease but without calciphylaxis. After a meticulous clinical chart review, we assessed the corresponding skin biopsies for the presence of vessel calcification, thromboses, and dermal angioplasia in skin biopsies from patients with calciphylaxis (n = 57) and compared with those from patients with end-stage renal disease but without calciphylaxis (n = 26). Histopathologic findings were correlated with clinical features such as chronic kidney disease, diabetes, or associated malignancy. Our results validated a prior observation that calciphylaxis was significantly more likely to show calcification of dermal vessels and diffuse dermal thrombi. This study reports the frequent finding of dermal angioplasia, a potential marker of chronic low-grade ischemia, as another frequent microscopic finding in calciphylaxis. Among cases of calciphylaxis, histopathologic changes in patients with chronic kidney disease were indistinguishable from those in patients without chronic kidney disease, thereby implying a final common pathogenic pathway in both uremic and nonuremic calciphylaxis. In future, larger, prospective studies may be useful in validating these findings.

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Risk factors and mortality associated with calciphylaxis in end-stage renal disease.

Alan C Breen, Sharon K. Davis, D. Gillen … (2001)

We conducted a case control study to determine risk factors and mortality associated with calciphylaxis in end-stage renal disease. Cases of calciphylaxis diagnosed between December 1989 and January 2000 were identified. Three controls were identified for each hemodialysis patient, with calciphylaxis matched to the date of initiation of hemodialysis. Laboratory data and medication doses were recorded during the 12 months prior to the date of diagnosis and at the time of diagnosis of calciphylaxis. Conditional logistic regression was used to identify risk factors for calciphylaxis. Cox proportional hazards models were used to estimate the risk of death associated with calciphylaxis. Nineteen cases and 54 controls were identified. Eighteen patients were hemodialysis patients, and one had a functioning renal allograft. Diagnosis was confirmed by skin biopsy in 16 cases. Women were at a sixfold higher risk of developing calciphylaxis (OR = 6.04, 95% CI 1.62 to 22.6, P = 0.007). There was a 21% lower risk of calciphylaxis associated with each 0.1 g/dL increase in the mean serum albumin during the year prior to diagnosis and at the time of diagnosis of calciphylaxis (OR = 0.79, 95% CI, 0.64 to 0.99, P = 0.037, and OR = 0.80, 95% CI, 0.67 to 0.96, P = 0.019, respectively). There was a 3.51-fold increase in the risk of calciphylaxis associated with each mg/dL increase in the mean serum phosphate during the year prior to diagnosis (95% CI, 0.99 to 12.5, P = 0.052). At the time of diagnosis of calciphylaxis, for each 10 IU/L increment in alkaline phosphatase, the risk of calciphylaxis increased by 19% (OR = 1.19, 95% CI, 1.00 to 1.40, P = 0.045). Body mass index, diabetes, blood pressure, aluminum, and higher dosage of erythropoietin and iron dextran were not independent predictors of calciphylaxis. Calciphylaxis independently increased the risk of death by eightfold (OR = 8.58, 95% CI, 3.26 to 22.6, P < 0.001). Female gender, hyperphosphatemia, high alkaline phosphatase, and low serum albumin are risk factors for calciphylaxis. Calciphylaxis is associated with a very high mortality.

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Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B.

Roger Weenig (2008)

The clinical syndrome of calciphylaxis is characterized by arteriolar medial calcification, thrombotic cutaneous ischemia, necrotic skin ulceration, and a high mortality rate. This review integrates calciphylaxis risk factors with the molecular processes governing osseous and extraosseous mineralization. As the pathogenesis of calciphylaxis is better understood, targeted therapies aimed at disease prevention and reversal will follow.

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Cutaneous calciphylaxis: a retrospective histopathologic evaluation.

Mark C Mochel, Ryan Arakaki, Guilin Wang … (2013)

Calciphylaxis is a rare and life-threatening disease characterized by cutaneous necrosis and vascular calcification. Often, skin biopsy specimens are not diagnostic because of the limited depth of the specimen, biopsy site, and clinical stage. To better understand the utility of various histologic features in rendering the diagnosis of calciphylaxis and to compare von Kossa versus Alizarin red stains in the detection of calcium deposits, we retrospectively analyzed the histologic features and histochemical stain findings of 56 skin biopsies from 27 consecutive patients seen at Massachusetts General Hospital from October 2002 to April 2012, with confirmed diagnosis of calciphylaxis and compared with that of 19 skin biopsies from 17 patients with other disease processes. All forms of vascular calcification and vascular thrombosis were significantly associated with cutaneous calciphylaxis. Perieccrine calcium deposition, highly specific to calciphylaxis, was the only form of calcium deposition noted in 4 (7%) skin biopsies from patients with calciphylaxis. Although the staining appears to be comparable, the deposits seen on Alizarin red appeared larger and were birefringent. Although subtle, perieccrine calcification may aid in the diagnosis of calciphylaxis in settings where typical vascular and extravascular calcification are not identified. Performing both von Kossa and Alizarin red stains might increase the detection of calcium deposit.