Calciphylaxis: seven new cases.

BACKGROUND

Calciphylaxis is a rare condition occurring most frequently in patients with end-stage renal disease (ESRD). It is characterized by vascular calcifications with a large variety of skin lesions. Even though this entity was first reported almost 50 years ago, the pathogenesis is still not well understood.

OBJECTIVE

Having retrieved seven new cases from a single tertiary care hospital, the disease occurs probably more frequently than reported until now. The potential mechanism of action in this disease is discussed, particularly the hypercoagulability state. We also review potential treatments described in the literature.

METHODS

Seven patients with calciphylaxis that occurred at the Hôtel-Dieu hospital between 1992 and 1998 were identified and their case histories reviewed and analyzed.

CONCLUSION

Although hyperparathyroidism and imbalance of calcium-phosphorus homeostasis are paramount for calciphylaxis to occur, other mechanisms must be involved because the disease manifests itself in only a minority of ESRD patients. As the majority were under anticoagulation therapy and as we found abnormalities of the coagulation pathway in one patient, we suggest emphasizing these phenomena in the future. Along with evaluation of putative risk factors (abnormalities of the calcium:phosphate axis, diabetes), a detailed evaluation of the coagulation system should be done in every patient with calciphylaxis until more data are available.