Calciphylaxis is characterized by progressive cutaneous necrosis associated with small- and mediumsized vessel calcification occurring in the setting of end-stage renal disease, diabetes mellitus, and hyperparathyroidism. Cutaneous involvement presents as initial painful geographic areas of ischemia that Progress to gangrene and ulceration of the subcutaneous fat, dermis, and epidermis; secondary infection and sepsis are common.
Causes of Calciphylaxis
The pathogenesis is poorly understood. In animal models, calciphylaxis is described as a condition of induced systemic hypersensitivity in which tissues respond to appropriate challenging agents with calcium deposition. Calciphylaxis is associated with chronic renal failure, secondary hyperparathyroidism, and an elevated calcium phosphate end product. Implicated “challenging agents” include glucocorticoids, albumin infusions, intramuscular tobramycin, iron dextran complex, calcium heparinate, immunosuppressive agents, and vitamin D.
Symptoms of Calciphylaxis
Patients usually experience unbearable pain, burning and sometimes itching at the lesion sites.
Calciphylaxis most often occurs on the lower limb especially in fatty areas. Lesions on the trunk, abdomen, buttocks or thighs, appear to be more dangerous than lesions on the lower legs and feet.
Diagnosis
Made on history of renal failure, clinical findings, elevated PTH level, elevated calcium X phosphate ion product, and histologic features.
Treatment
Calciphylaxis is best managed by early diagnosis, treatment of renal failure, partial parathyroidectomy when indicated, aggressive debridement of necrotic tissue and avoidance of precipitating factors such as systemic glucocorticoids.
Wound management is important.
- Keep wounds clean
- Surgically remove the necrotic tissue – sometimes this means a limb must be amputated
- Systemic antibiotics
References