Calciphylaxis in a Patient Without Chronic Kidney Disease: A Case Report and Review of the Literature

1Beth Israel Deaconess Medical Center, Boston, MA

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 257

Case Presentation:

This is a 65‐year‐old woman with history of Crohn's disease and prolonged surgical hospitalization 14 weeks prior to admission for perforated colon secondary to diverticulitis requiring lower anterior resection with end‐ileostomy. Her course was complicated by iatrogenic bladder laceration with repair and pulmonary embolism, for which she received warfarin. She improved and was transferred to a rehabilitation center. She was subsequently readmitted with urosepsis, for which she received antibiotics and heparin anticoagulation bridging back to warfarin. She was again discharged to rehabilitation. Four weeks after warfarin reinitiation she presented with painful skin lesions. Physical exam revealed morbid obesity and exquisitely tender, firm, indurated, violaceous plaques with areas of necrosis extending from the abdominal flanks to the proximal lateral thighs bilaterally. Laboratory analysis was notable for leukocytosis, INR 2.3, serum blood urea nitrogen 145 mg/dL, serum creatinine 3.4 mg/dL (baseline, 0.5 mg/dL), hypoalbuminemia, hypokalemia, hypercalcemia, hyperphosphatemia, and muddy brown urinary casts. Serum PTH was normal, protein S activity was decreased (26%), lupus anticoagulant was positive, and ANA and anticardiolipin antibodies were negative. The differential diagnosis included late‐onset warfarin‐induced skin necrosis, calciphylaxis, and catastrophic antiphospholipid antibody syndrome. Warfarin was discontinued, and vitamin K was administered. Skin biopsy showed subcutaneous vascular congestion and thrombosis with focal vascular calcification. Intravenous Sodium thiosulfate was initiated. Triplephase bone scanning with technetium‐99m methylene diphosphonate showed normal vascular flow and blood pool images but delayed radiotracer uptake in the subcutaneous tissues of the thighs, consistent with calciphylaxis. Sodium thiosulfate was continued thrice weekly, and the patient received pamidronate, sevelamer, and opiate pain medication. A low‐phosphate diet was initiated. Serum creatinine improved to 0.9 mg/dL with fluid resuscitation.

Discussion:

Calciphylaxis is a rare, life‐threatening condition and may be confused with other disorders causing skin necrosis. It almost always occurs in patients with end‐stage renal disease but can occur in acute renal failure and in the absence of kidney disease. Morbidity and mortality are estimated at 50%–80% and are the result of pain, opiate pain medication, and sepsis. Empiric treatment is often necessary. Sodium thiosulfate is a relatively new treatment option for calciphylaxis.

Conclusions:

The purpose of presenting this case is to increase awareness of this rare condition, which may occur in patients without chronic kidney disease, and to highlight its evaluation and treatment. To our knowledge, this is the third reported case of the use of sodium thiosulfate to treat calciphylaxis in a patient without chronic kidney disease.

Disclosures:

J. T. Crocker ‐ none; J. C. Hart ‐ none

To cite this abstract:

Crocker J, Hart J. Calciphylaxis in a Patient Without Chronic Kidney Disease: A Case Report and Review of the Literature. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 257. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/calciphylaxis-in-a-patient-without-chronic-kidney-disease-a-case-report-and-review-of-the-literature/. Accessed April 21, 2019.

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