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Diagnosing Calciphylaxis: A Review With Emphasis on Histopathology
Eman Bahrani1, Ifeoma U Perkins2, Jeffrey P North3
1Resident Physician, Department of Dermatology, University of California, San Francisco, CA.
Insights
Calciphylaxis, a severe skin vascular disease, presents diagnostic challenges due to subtle histopathologic features. This review correlates clinical and histopathologic findings with imaging and pathophysiology to aid diagnosis.
Area of Science:
- Dermatology
- Pathology
- Vascular Biology
Background:
- Calciphylaxis is a critical cutaneous vasculopathy associated with high morbidity and mortality.
- It involves vascular intimal fibrosis, calcification, stenosis, thrombosis, and ischemic tissue death.
- Histopathologic diagnosis is challenging due to subtle features and overlap with other conditions.
Purpose of the Study:
- To review and correlate clinical and histopathologic features of calciphylaxis.
- To integrate findings with imaging, ancillary studies, and pathophysiology.
- To identify diagnostic clues for calciphylaxis.
Main Methods:
- Review of reported clinical and histopathologic features of calciphylaxis.
- Correlation with imaging modalities and ancillary diagnostic studies.
- Examination of underlying pathophysiology.
Main Results:
- Histopathologic changes in calciphylaxis often overlap with Mönckeberg sclerosis, lupus panniculitis, pancreatic panniculitis, and peripheral artery disease.
- Key diagnostic clues include subcutaneous small vessel calcification, thrombosis, ischemic changes, pseudoxanthoma elasticum-like changes, and perieccrine calcification.
- Special stains (von Kossa, Alizarin red) and imaging (netlike calcification) can aid in identifying calcification.
Conclusions:
- While challenging, specific histopathologic and imaging features can support calciphylaxis diagnosis.
- Ongoing research into calciphylaxis pathophysiology may yield improved diagnostic adjuncts.
- Increased diagnostic sensitivity and specificity are needed for this severe condition.
Abstract:
Calciphylaxis is a cutaneous vasculopathy with high morbidity and mortality characterized by vascular intimal fibrosis, calcification, stenosis, thrombosis, and eventual tissue death due to ischemia. Histopathologic diagnosis is often difficult, frequently necessitating multiple tissues samples due to lack of specific histopathologic features and subtle changes on biopsies of early lesions. In this study, we review the reported clinical and histopathologic features of calciphylaxis, correlating them with relevant imaging, ancillary studies, and pathophysiology. Although many histopathologic changes seen in calciphylaxis are also reported in other conditions (eg, Mönckeberg sclerosis, lupus panniculitis, pancreatic panniculitis, and peripheral artery disease), calcification of subcutaneous small vessels, thrombosis and/or ischemic changes, pseudoxanthoma elasticum-like changes in the subcutis, and perieccrine calcification may serve as helpful clues. von Kossa and Alizarin red stains can assist in the identification of subtle calcification. Netlike calcification of the affected blood vessels on imaging further supports the diagnosis. Studies into the pathophysiology of calciphylaxis are ongoing and will hopefully facilitate the development of additional diagnostic adjuncts to increase sensitivity and specificity for the diagnosis of this disease.
