Dr. Vincent DeLeo talks to Dr. Robert G. Micheletti about managing patients with calciphylaxis, a rare but potentially fatal condition classically seen in patients with end-stage renal disease (ESRD). Early recognition and diagnosis of calciphylaxis are essential to reducing morbidity and mortality. Dr. Micheletti describes the clinical features of calciphylaxis that dermatologists may encounter bedside, noting that biopsy often is nondefinitive.
“It’s a tough disease to have," Dr. Micheletti explains, "which is why you do need multidisciplinary care and the help of a good dermatologist to be able to make the diagnosis and address the wound situation.”
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Things you will learn in this episode:
- Although calciphylaxis commonly is associated with ESRD, nonuremic calciphylaxis can be triggered by other clinical factors in a subset of patients without ESRD.
- Risk factors for calciphylaxis include various medications, clotting disorders, and autoimmune diseases, whether the patient also has ESRD.
- The clinical presentation of calciphylaxis depends on the point at which the area is examined. Early stages of calciphylaxis may present as a tender subcutaneous nodule, while late stages may present with more severe pain and ulceration. Maintain a high index of suspicion for calciphylaxis in patients with ESRD on chronic dialysis presenting with severely painful livedoid plaques or retiform purpura, particularly in fat-rich body sites.
- Biopsy often is nondiagnostic because of insufficient tissue sample size. Calcium stains will help highlight areas of vascular calcification, but “don’t assume just because the biopsy doesn’t show calcification that it is not calciphylaxis.” To improve diagnostic accuracy, biopsy specimens should be evaluated by experienced dermatopathologists who have seen calciphylaxis before.
- End-stage renal disease patients with calciphylaxis who are not currently on dialysis may benefit from starting it.
- Dermatologists should work in conjunction with nephrologists to optimize dialysis and other medications to treat underlying issues associated with calciphylaxis in the setting of ESRD.
- Data-driven diagnostic criteria and management guidelines for calciphylaxis are needed to improve patient care. The Society for Dermatology Hospitalists is working on pooling cases of calciphylaxis to generate a data-driven model of factors associated with the diagnosis.
Hosts: Elizabeth Mechcatie; Terry Rudd; Vincent A. DeLeo, MD (University of Southern California, Los Angeles).
Guest: Robert G. Micheletti, MD (Departments of Dermatology and Medicine, University of Pennsylvania, Philadelphia).
Show notes by Alicia Sonners, Melissa Sears, and Elizabeth Mechcatie.
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