Severe, sometimes fatal disorder
- Mechanism
- Metastatic calcification in blood vessels
- Cutaneous ischemia and necrosis
- Etiology: due to disturbances in calcium homeostasis
- 2 major forms
- Uremic (end stage renal disease, on hemodyalisis, with secondary hyperparathyroidism)
- Non-uremic (other medical conditions or medications)
- Warfarin, high dose steroids
- Primary hyperparathyroidism
- Ectopic PTH secretion by carcinoid tumor
- Vitamin D intoxication
- Fatores de risco
- hypercoagulability: underlying protein C disfunction, antiphospholipid antibodies
- obesity
- Clinical presentation
- Markedly painful retiform purpura and ulcerations
- Cutaneous and subcutaneous calcifications - plaques or nodules
- Underlying hard, stone-like induration
- Distribution Sometimes linear
- Sometimes retiform purpura/livedoid appearance
- PAIN
- Reticulated violaceous plaques with central necrosis or echar
- Sometimes, retiforme purpura or bullae precede the ulceration
- Exames complementares de diagnĂłstico
- Ca*PO4 product may be elevated
- Can use imaging (XRay, CT, U/S or scintigraphy), if biopsy unobtainable
- Review of recently performed radiology may uncover “missed” diagnoses
- Histology
- Padrão vasculopático
- Calcification of vessel walls leading to ischemia
- Ischemic fat necrosis and mixed cell infiltrate
- Calcium deposits visible with Von Kossa
- Deposits of calcium within blood vessel walls of the subcutis usually present on skin biopsy
- Tratamento
- TOC: IV Sodium thiosulfate (chelator) 25g 3x/semana, continued for 2 months after healing
- Treatment
- Avoid warfarin and prednisone → known triggers
- Low calcium dialysis
- Phosphate binders
- Parathyroidectomy
- Aggressive wound care
- Sodium thiosulfate local injections
- Pamidronate
- Cinacalcet
- Apixaban
- 1 year mortality ~50% → poor prognosis
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