Calciphylaxis is a poorly-understood condition whose pathogenesis involves systemic calcification of arteries and arterioles. It is usually seen in patients end-stage renal disease, with an incidence of approximately 5% in dialysis patient and patients with calcium-phosphate dysregulations.1,2 However, there have also been reports of patients with biopsy-proven calciphylaxis with normal calcium-phosphate balance and renal function. We report a morbidly obese 45-year-old female with significant past medical history of necrotizing fasciitis with superimposed pseudomonas infections requiring multiple rounds of antibiotics and debridement. She presented to hospital due to chronic thigh wounds and debilitating pain. Patient developed tender and ulcerated lesions on her bilateral inner thighs spontaneously and was treated with trimethoprim-sulfamethoxazole and doxycycline. Wound cultures grew pseudomonas and Methicillin-Resistant Staphylococcus aureus. Rheumatologic work up including antinuclear antibody, rheumatoid factor, anti-double stranded DNA, anti-ribonucleoprotein and complement levels were all within normal limits except for elevated erythrocyte sedimentation rate and c-reactive protein. Patient was given multiple analgesics of which ketorolac helped the most. She was referred to dermatology after which excisional biopsy of wound was performed. Biopsy result revealed tissue necrosis and calciphylaxis. Patient was started on sodium thiosulfate (STS) infusions after discussing with dermatology and was discharged in stable conditions from hospital. The exact cause of calciphylaxis still remains unknown. It is thought to be due to intravascular calcium deposition in the media of the epidermal and subcutaneous arterioles causing medial calcification and intimal fibrosis of the arterioles resulting in thrombosis and occlusions. This leads to ischemic skin necrosis which is the most common clinical finding in calciphylaxis.3 For non-uremic calciphylaxis, there appears to be a predilection of Caucasian females, primary hyperparathyroidism, obesity, malignancy, connective tissue disease and vitamin D deficiency.4-5 Our patient had some of the risk factors including morbid obesity, middle aged Caucasian female and Vitamin D deficiency. Calciphylaxis has two-year mortality rate of 50-80% secondary to sepsis, hence preventing patients with known risk factors from developing calciphylaxis is imperative.6 The lesions of calciphylaxis are often debilitating and wound care with debridement of necrotic tissue as well as systemic antibiotics are of utmost importance, if indicated. In recent years, treatment include the use of STS, which chelate calcium from tissue deposits and bisphosphonates which are thought to help in removing arterial calcifications.7 It is important to understand that calciphylaxis may occur in patients without renal impairment and early interventions may be helpful to decrease debilitation and mortality.
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1196 |
| Date | 05 April 2018 |
| Creators | Arif, Sarah, M.D., ali, Muazzam, MD, Zhang, Michael, MD, Obeng, George, MD, masood, Sara, MD, Sriramoju, Vindhya, MD, Hannan, Abdul, MD, Goldstein, Jack, MD |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Format | application/pdf |
| Source | Appalachian Student Research Forum |
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