Case Presentation:
Calciphylaxis is a rare cause of skin necrosis due to calcium deposition within the vasculature, most commonly seen in patients with end stage renal disease. A 54-year-old male was transferred from a long-term acute care facility for evaluation of a 3 week history of bilateral proximal lower-extremity wounds. His past medical history was notable for hypertension, a seizure disorder, and alcoholic liver disease. The first wound started on the lateral aspect of his right thigh and was similar in appearance to an eschar. He was transferred from the aforementioned facility to a community hospital, where he underwent surgical debridement. His original surgical pathology was concerning for a possible vasculitic cause with chronic inflammatory changes, as well as, a report of calcium deposits that raised the suspicion of calciphylaxis. He was trialed on a course of systemic corticosteroids without improvement. His wound cultures were positive for Klebsiella pneumoniae and vancomycin-resistant Enterococcus. He was started on an appropriate antibiotic regimen 12 days after his initial surgical debridement. When the patient developed a similar wound on the medial aspect of his left thigh, he was transferred to our tertiary care center for further evaluation of his wounds. A punch biopsy of the right medial thigh eschar was performed and evaluated by our Dermatopathology colleagues. The biopsy confirmed the finding of calciphylaxis despite his history of a preserved estimated glomerular filtrate rate. His lab work was notable for a normal PTH, calcium, and phosphate. Once the pathological diagnosis was made, he was started on sodium thiosulfate. Supportive therapy with local wound care continued, and daily high-flow supplemental oxygen therapy was initiated.
Discussion:
Calcific uremic arteriolopathy (CUA) in non-uremic patients, non-uremic calciphylaxis (NUC), is an exceedingly rare case of non-healing necrotic wounds. The most up to date systematic review has identified 35 unique cases reported between 1956 and 2008. In patients with preserved renal function the most common patient population is Caucasian females between the ages of ­­15 and 82. The most common associated conditions in non-uremic patients includes hyperparathyroidism, malignancy, and alcoholic liver disease. There has also been association with preceding systemic steroid treatment. Liver disease was confirmed based on our patient’s thrombocytopenia, elevated INR (Protime), and abdominal ultrasound findings. Diagnosis is confirmed through biopsy and pathology. Hemotoxylin and eosin stained sections of subcutaneous tissue from the right medial thigh of our patient demonstrates prominent concentric mural calcifications of subcutaneous small vessels, adjacent fat necrosis, and vascular congestion consistent with the diagnosis of calciphylaxsis (Figure 1). This congestion and subseuqent narrowing of vascular lumens leads to the prominent epidermal necrosis and sloughing.
Conclusions:
Our case demonstrates the possibility of calicphylaxis in patients with no prior history of chronic kidney disease or end stage renal disease. The diagnosis is ultimately confirmed based on pathology. The treatment for CUA and NUC remains relatively empiric with limited objective research data. The mortality remains high despite appropriate therapy and is usually due to sepsis. Early recognition is critical as is a multi-disciplinary approach to care with appropriate antibiotics, wound care, and surgical care.
