Synopsis
Recognise painful calcific arteriolopathy before black necrosis appears, distinguish it from infection and other retiform disease, and coordinate urgent renal, wound, analgesic, metabolic and infection management despite limited treatment evidence.
- Calciphylaxis is calcific uraemic arteriolopathy: an uncommon, intensely painful occlusive small-artery disease seen mainly in advanced CKD and dialysis, although non-uraemic cases occur.
- Deep burning pain and firm woody subcutaneous induration can precede visible change; lesions evolve through mottled livedoid or retiform purple-grey plaques to ulcer and black eschar.
- On brown or black skin, early colour may be deep violet, grey-brown or subtle; exquisite focal pain, hardness, temperature and an angular vascular outline should trigger examination under good light and touch.
- Risk rises with dialysis duration, diabetes, obesity, female sex, hypoalbuminaemia, severe CKD-mineral disorder, warfarin, calcium loading, injection trauma and systemic inflammation, but normal calcium or phosphate does not exclude disease.
- Diagnosis is clinical and multidisciplinary; there is no perfectly sensitive gold-standard test. Deep edge biopsy can show calcified thrombosed arterioles but may cause a non-healing ulcer, infection or false-negative result.
- First-line action is not one drug: control pain, search for infection, optimise dialysis and calcium–phosphate–PTH management, review promoting medicines and deliver expert wound care concurrently.
- Sodium thiosulfate is widely used off-label, commonly after haemodialysis, but controlled efficacy evidence remains uncertain and metabolic acidosis, hypotension, nausea, sodium load and QT effects require monitoring.
- Review warfarin urgently with the anticoagulation indication owner; do not stop it unsafely or assume substitution is straightforward in mechanical valves or high-risk APS.
- Debridement is individualised: infected wet necrosis may need surgical source control, whereas dry stable eschar with poor perfusion can be harmed by reflex excision.