Calciphylaxis: Difference between revisions
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==Background== | ==Background== | ||
*Most commonly seen in ESRD patients on hemodialysis (~1%) | {{Skin anatomy background images}} | ||
*Most commonly seen in [[ESRD]] patients on [[hemodialysis]] (~1%) | |||
**Seen almost exclusively in patients with Stage 5 chronic kidney disease | **Seen almost exclusively in patients with Stage 5 chronic kidney disease | ||
*No available data in general population (non-uremic calciphylaxis) | *No available data in general population (non-uremic calciphylaxis) | ||
*Calcium and phosphate levels rise beyond solubility and precipitate in arteries | *[[hypercalcemia|Calcium]] and [[hyperphosphatemia|phosphate]] levels rise beyond solubility and precipitate in arteries | ||
*May be increasing due to widespread IV vitamin D | *May be increasing due to widespread IV vitamin D | ||
*Mortality as high as 60-80%; sepsis from necrotic skin lesions | *Mortality as high as 60-80%; [[sepsis]] from necrotic skin lesions | ||
==Clinical Features== | ==Clinical Features== | ||
*Very painful lesions develop suddenly and progress rapidly | *Very painful [[rash|lesions]] develop suddenly and progress rapidly | ||
* | *Dermatologic appearances: | ||
**Livedo reticularis | **Livedo reticularis | ||
**Stellate purpura | **Stellate purpura | ||
**Usually | **Usually lower extremities, hands, or torso | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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{{ESRD Associated Skin Conditions}} | {{ESRD Associated Skin Conditions}} | ||
== | ==Evaluation== | ||
===Labs=== | ===Labs=== | ||
*Serum PTH level | *Serum PTH level | ||
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===Imaging=== | ===Imaging=== | ||
*Plain radiographs - arborization of vascular calcification within dermis and subQ tissues | *Plain radiographs - arborization of vascular calcification within dermis and subQ tissues | ||
*Ultrasound may aid in examining for vascular calcification<ref>Bukhman R et al. Sonography in the Identification of Calciphylaxis of the Breast. JUM January 1, 2010 vol. 29 no. 1 129-133.</ref> | |||
===Biopsy<ref>Nigwekar SU et al. Calciphylaxis: Risk Factors, Diagnosis, and Treatment. Am J Kidney Dis. 2015;66(1):133-146.</ref>=== | |||
*Definitive means of diagnosis | |||
*Punch biopsy from lesion margin by dermatologist or wound surgeon | |||
*Caution in non-ulcerated/necrotic lesions as biopsy site has high likelihood of not healing in true calciphylaxis | |||
==Management== | ==Management== | ||
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**Calcimimetics in hyperparathyroidism | **Calcimimetics in hyperparathyroidism | ||
**Bisphosphonates | **Bisphosphonates | ||
**Sodium thiosulfate - off-label, increases solubility of calcium deposits | **[[Sodium thiosulfate]] - off-label, increases solubility of calcium deposits | ||
**Fix hypercoagulability | **Fix hypercoagulability | ||
**Surgical | **Surgical | ||
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[[Category:FEN]] | [[Category:FEN]] | ||
[[Category: | [[Category:Dermatology]] | ||
Latest revision as of 16:18, 11 December 2024
Background
- Most commonly seen in ESRD patients on hemodialysis (~1%)
- Seen almost exclusively in patients with Stage 5 chronic kidney disease
- No available data in general population (non-uremic calciphylaxis)
- Calcium and phosphate levels rise beyond solubility and precipitate in arteries
- May be increasing due to widespread IV vitamin D
- Mortality as high as 60-80%; sepsis from necrotic skin lesions
Clinical Features
- Very painful lesions develop suddenly and progress rapidly
- Dermatologic appearances:
- Livedo reticularis
- Stellate purpura
- Usually lower extremities, hands, or torso
Differential Diagnosis
- Brown recluse spider bite
- Bullous pemphigoid
- Cellulitis, necrotizing fasciitis
- Erythema nodosum
- Vasculitis
- Venous ulcers
- Hypercalcemia
- Hyperphosphatemia
ESRD Associated Skin Conditions
- Calciphylaxis
- Nephrogenic Systemic Fibrosis (gadolinium MRI)
Cardiovascular
Evaluation
Labs
- Serum PTH level
- CBC, CMP, phosphate, coags
- Inpatient - hepatitis panel, cryofibrinogen level, lipase, ESR, CRP, ANA, ANCA
Imaging
- Plain radiographs - arborization of vascular calcification within dermis and subQ tissues
- Ultrasound may aid in examining for vascular calcification[1]
Biopsy[2]
- Definitive means of diagnosis
- Punch biopsy from lesion margin by dermatologist or wound surgeon
- Caution in non-ulcerated/necrotic lesions as biopsy site has high likelihood of not healing in true calciphylaxis
Management
- Rigorous and continuous control of phosphate and calcium balance
- Medical
- Discontinue calcium increasing interventions
- Increase dialysis frequency
- Calcimimetics in hyperparathyroidism
- Bisphosphonates
- Sodium thiosulfate - off-label, increases solubility of calcium deposits
- Fix hypercoagulability
- Surgical
- Aggressive wound care and debridement of necrotic tissues
- Wound VAC
- Total or subtotal parathyroidectomy
Disposition
- Admit