Skin & Soft Tissue: Calciphylaxis (Calcific Uremic Arteriolopathy)

Calciphylaxis (Calcific Uremic Arteriolopathy)

Basics

  • Skin Ischemia & Necrosis with Arteriole/Capillary Calcification
  • Pathogenesis Poorly Understood
  • Poor Prognosis
    • 6-Month Survival: 50%

Risk Factors

  • End-Stage Renal Disease – Strongest Risk Factor
  • Hyperphosphatemia
  • Warfarin
  • Female Sex
  • Obesity
  • Diabetes

Affected Sites

  • Most Common in Areas of Increased Adiposity
  • Distal Lower Extremity (55% – Most Common)
  • Proximal Lower Extremity (39%)
  • Trunk (31%)
  • Distal Upper Extremity (7%)
  • Proximal Upper Extremity (3%)

Presentation

  • Early Lesions: Exquisitely Painful Areas of Reddish-Blue Induration, Plaque & Subcutaneous Nodules
  • Later Lesions: Ischemic/Necrotic Ulcers with Eschar

Calciphylaxis 1

Diagnosis

  • May Be Made on Classic History & Exam
  • Biopsy Can Confirm if Uncertain
  • Histology:
    • Dermo-Hypodermal & Pannicular Arteriolar Calcification
    • Subintimal Fibrosis
    • Thrombotic Occlusion

Treatment

  • No Good Treatment
  • General Management: Wound Care (Dressings, Debridement & Negative-Pressure Wound Therapy)
  • May Require Antibiotics & Debridement if Infected

Calciphylaxis Histology (Arteriolar Calcification) 2

References

  1. Marques SA, Kakuda AC, Mendaçolli TJ, Abbade LP, Marques ME. Calciphylaxis: a rare but potentially fatal event of chronic kidney disease. Case report. An Bras Dermatol. 2013 Nov-Dec;88(6 Suppl 1):44-7. (License: CC BY-NC-3.0)
  2. Miura S, Takahashi K, Akasaka T. Calciphylaxis Presenting with Various Symptoms: A Case Report. Case Rep Dermatol. 2017 Feb 8;9(1):25-29. (License: CC BY-NC-4.0)