A 48-year-old morbidly obese woman with history of sarcoidosis with annual cutaneous flares was admitted for extensive non-healing, necrotic ulcers on her lower extremities. Three months prior to presentation, the patient developed open ulcers on her legs which she attributed to a typical sarcoid flare. She later developed diffuse body malaise and dark urine. She presented to an outside ED and was found to be in septic shock. She required intensive care for three weeks, with course complicated by acute renal failure requiring temporary dialysis. Her kidney function recovered to normal by discharge. She was soon readmitted with new, expanding bullae on her right hip, bilateral thighs and left foot. Skin biopsy was nondiagnostic. She was treated with high-dose steroids without improvement and transferred to our institution. She was clinically diagnosed with calciphylaxis, later confirmed on repeat biopsy. She was rapidly tapered off of steroids and started on sodium thiosulfate. Her course was notable for expanding necrosis and development of new ulcers. She also suffered recurrent superinfections by progressively drug-resistant organisms. Given her complex wound care and the challenging pain-control issues, the patient was ultimately transferred to a specialized burn unit.
Discussion:
Calciphylaxis, or calcific uremic arteriolopathy, is a devastating disease characterized by painful skin necrosis, usually in areas of adiposity. While the exact pathogenesis is not completely understood, it is a consequence of obstructive vascular calcification, most commonly seen in end-stage renal disease. Diagnosis is largely clinical; skin biopsy can be useful in atypical cases, but sensitivity is limited. The classic elevation in calcium x phosphorus product is supportive but often absent, as was the case with our patient. Sodium thiosulfate has gained increasing popularity as treatment and is felt to act via multiple mechanisms including chelation, antioxidant effect, and vasodilation. Calciphylaxis carries a high mortality rate due to infectious complications, and thus wound care and debridement are essential cornerstones of therapy.
Conclusions:
This case highlights an unusual presentation of a devastating disease. Non-uremic calciphylaxis is reported in the literature but remains exceedingly rare and thus poorly understood. This case suggests that even transient renal failure may play a role in the development of calciphylaxis. The patient’s sarcoidosis and prolonged exposure to glucocorticoids may also have contributed, if not to the instigation, then perhaps to the protracted and refractory course of the disease, although rheumatologic conditions associated with calciphylaxis are generally thought to be limited to the connective tissue diseases. The cataloguing of unique cases such as this one can hopefully deepen our understanding of this morbid diagnosis.