09/21/2021
Diagnosis of Calciphylaxis
Diagnosis can be made based on clinical symptoms, but patients may benefit from further testing and laboratory work to evaluate for an underlying condition that may have contributed to the development of a calciphylaxis wound.
Testing includes the following:
• Biopsy: look for calcification, microthrombosis, and fibrointimal hyperplasia of dermal or subcutaneous vessels2
• Laboratory tests to assess for risk factors2:
◦ Blood urea nitrogen-to-creatinine ratio and glomerular filtration rate
◦ Serum calcium, phosphorus, alkaline phosphatase, parathyroid hormone, 25-hydroxyvitamin D
◦ Liver testing: transaminase, alkaline phosphatase, and albumin
◦ Evaluation for infection: complete blood count with differential
◦ Coagulation evaluation: partial thromboplastin time
Clinical Manifestations
Clinical features include the following:
• Painful ulcers
• Lacy pattern (livedo reticularis), which evolves to larger plaques and eventually black eschar
• Wound on areas with more subcutaneous fat, likely on the trunk and lower extremities3
• High mortality rate, with sepsis the leading cause of death1
Diagnosis
To determine if you have calciphylaxis, your doctor will review your medical history, assess your symptoms and do a physical exam. Tests may include:
• Skin biopsy. To diagnose calciphylaxis, your doctor may remove a small tissue sample from an area of affected skin for analysis.
• Blood tests. Blood samples measure a variety of substances in your blood — calcium, phosphorus, parathyroid hormone, abnormalities in blood-clotting factors, aluminum, urea nitrogen, creatinine and albumin, among others — to help your doctor assess your kidney and liver function.
• Imaging studies. X-rays may show branch-like calcium deposits in the blood vessels (vascular calcifications) that are common in calciphylaxis and in other advanced kidney diseases.
Unfortunately, even with appropriate treatment, calciphylaxis remains extremely deadly. The estimated 1-year survival rate in one study was 45.8%; more than half of deaths are caused by infection. Although some patients treated with a multi-modality approach as described above can see improvement and even disappearance of lesions, they nevertheless suffer from high morbidity due to pain, immobility and frequent medical interventions. Overall prognosis seems to be better if calciphylactic lesions are treated early in their development, stressing the importance of recognizing this rare condition and prompt treatment of any associated skin and soft tissue infections.
NSAIDs help ease pain and inflammation. But if you have high blood pressure, heart failure, or kidney disease, you should not take an NSAID. And you should not take any drugs that have ibuprofen or another NSAID in them.
Conclusion
Once the appropriate interventions have been made, frequent follow-up is essential to ensure that the therapy is working and the wounds are improving. Calciphylaxis is an uncommon condition but one that is treatable. With the proper sleuthing and the right care, these wounds can be healed. Understanding that these wounds are like "a heart attack of the skin" can guide you in the right direction and help put your loved one on the path to recovery.
