Diagnosing Calciphylaxis: A Review With Emphasis on Histopathology

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.