Case Presentation: A 60 year-old man with a past medical history of end-stage renal disease (ESRD) on hemodialysis (HD) was admitted after a becoming hypotensive during dialysis. The patient denied any fevers or chills, but endorsed progressive malaise and increased pain on the tip of his penis. Physical exam was significant for a blood pressure of 84/56 mmHg, heart rate of 92 bpm, and a tender black eschar on the tip of the glans with surrounding erythema and purulent drainage. Laboratory data was notable for hypercalcemia of 13.3 mg/dl and hyperphosphatemia of 6.7 mg/dl. He was started on intravenous fluids and broad-spectrum antibiotics with concern for calciphylaxis with secondary infection. Dermatology and urology were consulted for gangrene and concurred with the diagnosis of penile calciphylaxis. Given the active infection, a biopsy was not pursued. Despite a three week course of antibiotics and sodium thiosulfate, the patient did not tolerate HD due to low blood pressures. Given his poor overall prognosis due to penile calciphylaxis, the patient elected to pursue comfort measures and was transferred to inpatient hospice.
Discussion: Calciphylaxis is a rare and life threatening condition most commonly observed in patients with ESRD on dialysis. Calciphylaxis is seen in 1-4% of patients with ESRD. It is characterized clinically by areas of painful ischemic necrosis and histologically by calcification of arterioles in both the dermis and subcutaneous adipose tissue. ESRD patients are especially at risk for calciphylaxis due to alterations in arterial blood flow from calcification and fibrosis of arterioles. Patients are often found to have concurrent hypercalcemia and hyperphosphatemia. While calciphylaxis is sometimes a clinical diagnosis, the gold standard remains skin biopsy. Calciphylaxis is typically seen in a proximal distribution, often affecting the thighs, abdominal wall, and buttocks. There have been few reported incidences of isolated penile calciphylaxis. Providers must be aware of atypical presentations of calciphylaxis in other areas, such as the genitals, as seen in our patient. Penile calciphylaxis carries a high mortality rate, ranging from 60% to 69% within 6 months, often as a result of infection from gangrenous tissue. While the optimal therapy for calciphylaxis is unknown, the majority of ESRD patients are empirically treated with sodium thiosulfate (STS). Although STS is used frequently in clinical practice, clinicians must be aware that its efficacy is sub-optimal in patients with penile calciphylaxis.
Conclusions: Calciphylaxis is a life-threatening condition that predominantly affects patients with end-stage renal disease on hemodialysis. Hospitalists must maintain a high index of clinical suspicion when caring for patients with apparent necrosis of the genitals to allow for early diagnosis of calciphylaxis, as this could yield more a more accurate long-term prognosis.