Abstract: SA-PO934

A Rare Case of a Large Calcified Mass in a Calciphylaxis Patient

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Patil, Raju A., Emory University, Decatur, Georgia, United States
  • Cobb, Jason, Emory University School of Medicine, Atlanta, Georgia, United States
  • Daring, Peter, Colorado College, Decatur, Georgia, United States
  • Gray, Carol A., Emory Healthcare, Atlanta, Georgia, United States
  • Mckinnon, William, Peachtree Vascular Surgery, Atlanta, Georgia, United States
Background

Calciphylaxis is a rare clinical disorder in patients with ESRD and is characterized by painful skin ulcerations due to ischemia and necrosis of the skin and subcutaneous adipose tissue. The disorder carries a mortality rate of 80% in the first year, and death is often due to recurrent infections leading to sepsis. The pathogenic mechanism is not clearly understood and therapeutic options are limited. We present a rare case of a calcified tumor from a patient suffering from calciphylaxis measuring 23.0 x 22.0 x 6.5 cm and 27 x 26 x7.5 cm on the medial aspect of the thigh.

Results

A 58 year old black female with ESRD due to a collapsing glomerulopathy, diabetes, developed symptoms of pain and skin ulcerations over her bilateral lower extremities and abdomen. A tissue biopsy of the lesions revealed necrotic ulcerations of the epidermal and dermal layers, and focal calcium deposition in the subcutaneous adipose tissue. She presented to the hospital with symptoms of fever, weakness and pain, with large areas of induration and skin ulcerations (the largest measuring 20 cm) over the inner aspects of the thigh bilaterally. Examination revealed a large area of induration and skin ulcerations with a large firm mass. Lab levels included PTH <100 pg/ml (she presented on cinacalcet), serum phosphorus within normal limits, and unfortunately she had been on long-term warfarin due to chronic dialysis access thrombosis. She was transferred to our hospital for further management of calciphylaxis which included wound care, surgery debridement, and hyperbaric oxygen therapy. Vascular surgery excised 27 x 26 x 7.5 cm and 23.0 x 22.0 x 6.5 cm masses from the right and left inner thigh. Histopathology examination confirmed vascular calcifications, fat necrosis with fibrosis, inflammation, and subcutaneous calcification classically described in calciphylaxis. Approximately one month after surgery the patient subsequently succumbed to sepsis.

Conclusion

Although calciphylaxis most commonly presents with skin ulcerations and calcified deposition under the skin, this is an unusual case of calciphylaxis which manifested in the form of a large calcified mass. This is rarely described in the literature and one of the first cases of an excised mass with a pathological diagnosis. The pathogenesis of this proliferation of tissue in calciphylaxis needs to be further identified and studied.