Abstract Introduction Calciphylaxis is a well-known complication of critical illness, particularly in the setting of renal replacement therapy. Prognosis is poor, with 1 year mortality generally above 50%, and response to therapy is limited. In the ICU population, coagulopathy, chronic wounds, and bacterial infections can make diagnosis challenging. We present a case of presumptive calciphylaxis with a negative biopsy, leading to alternate diagnoses. Case A 38-year-old woman with history of spina bifida and neurogenic bladder presents to the hospital due to 5 days of progressive encephalopathy and concerns for systemic infection. On presentation, extremity exam notable for multiple areas of suspected skin infection on back and bilateral legs, and skin breakdown on left thigh. On admission, she was hypotensive with lab derangements notable for marked leukocytosis (55.7), serum creatinine of 12.1, potassium of 6.4, and a lactate of 5.1. She received IV fluids and empiric broad-spectrum antibiotics, however she developed worsening encephalopathy requiring intubation and kidney injury requiring renal replacement therapy. Over her ICU course, she had significant clinical improvement with respect to her shock and encephalopathy however there was progression of her wounds with development of raised borders, expanding peri-wound pallor, and further necrotic change. Based on evaluation by dermatology service, in collaboration with general surgery and wound care, these wound were found to be clinically consistent with calciphylaxis.Ultimately, she received wound debridement in the OR. Pathology from intra-operative biopsy demonstrated skin with extensive necrosis and organizing intravascular thrombi, little associated inflammation and negative stain for calcium (von Kossa), consistent with hypercoagulable disorder and less consistent with calciphylaxis. Hypercoagulable workup was negative, and presumptive diagnosis of thrombosis secondary to sepsis was established. Over the coming weeks wounds demonstrated remarkable improvement, and one month after presentation wounds were closed in the OR after significant improvement in margins and notable tissue healing. Discussion In a young patient with minimal past medical history and otherwise improving course of treated septic shock, the diagnosis of calciphylaxis would transform her course from one of recovery to terminal illness. This case demonstrates the challenges in establishing the diagnosis of calciphylaxis, along with the utility of tissue biopsy and monitoring of clinical course prior to establishing this diagnosis. Biopsy is not always recommended in cases of presumptive calciphylaxis, however in this case tissue biopsy results significantly modified the diagnostic course and ultimately led to the correct diagnosis of hypercoagulability secondary to sepsis. This abstract is funded by: None
Dahl et al. (Fri,) studied this question.