Introduction: Calciphylaxis, small vessel vasculopathy, is a rare, but serious disorder that usually occurs in a patient with End-stage renal disease (ESRD). It is characterized by the deposition of calcium in the arteriole's intimal layer with the consequent proliferation, fibrosis, and thrombosis of that layer. The diagnosis requires high clinical suspicion, as it usually presenting as atypical painful skin nodules or subcutaneous hard mass, or in advance cases, can present as an ischemic ulcer. This disorder is known to be associated with ESRD, however, rarely it starts before ESRD.We hereby, presenting a case of calciphylaxis that was missed for around two years, as it presented Atypically in a patient weeks before becoming ESRD.
Aim:The aim of this study is to increase physician awareness about this rare medical condition and to highlight the possibility of this disease starting before the patient's renal insufficiency advanced to the ESRD level.Case report: An 82-year-old female with a Body mass index (BMI) of 30 and a past medical history of ESRD on home hemodialysis, insulin-dependent diabetes mellitus, presented with an event of fall. Her review of the systems showed that she is been complaining of subcutaneous hard and tender nodules in her lower extremities, mainly thighs and below knees, for more than 2 years. On examination: she had painful hard subcutaneous nodules that was movable and with no ulcer/ redness or fluctuation. Her labs showed elevated BUN and creatinine along with Hyperphosphatemia. Computed tomography (CT) scan and bone scan were consistent with calciphylaxis. This calciphylaxis was started before the peritoneal dialysis 2 years ago and was not ruptured or ulcerate at any time. The diagnosis of calciphylaxis was made clinically given the history of these painful nodules and associated risk factors of Hyperphosphatemia, Obesity, Female and Diabetic Mellitus. And was strengthened by the CT scan and bone scan findings. Conclusion: Calciphylaxis (calcific uremic arteriolopathy) is a rare but serious disorder that usually occurs in patients with ESRD but can occur in patients without ESRD or before the ESRD. The diagnosis can be based on clinical grounds and supported by excluding another differential diagnosis, ESRD-associated labs if present along with high phosphate and calcium can also support the diagnosis. Imaging and histological analysis, if possible, can also help and support the diagnosis. All the physicians and mainly (Dermatologist and PCP) must have a high level of awareness of this condition as the early detection and treatment can help in improving the outcome.