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Calciphylaxis

Title               

Ñ    Calciphylaxis

Definition

A syndrome of:

Ñ    Vascular calcification and skin necrosis

Ñ    Associated with increased morbidity

Ñ    Pathogenesis poorly understood

Pathogenesis

Ñ    Poorly understood

Ñ    Associated with chronic renal failure, hypercalcaemia, hyperphosphataemia, elevated calcium-phosphate product and secondary hyperparathyroidism

Ñ    Although these features are common in end stage renal failure; calciphylaxis is rare

Ñ    Vascular calcification is a constant finding in calciphylaxis

The theoretical roles played by vascular calcification are:

·          Calcification of the vascular endothelium results in a microenvironment that predisposes to hypercoagulability

·          Extensive endothelial calcification and intimal hyperplasia may result in vascular occlusion

Ñ    Calciphylaxis has also been reported in patients with primary hyperparathyroidism, cirrhosis, rheumatoid arthritis

History

Sex

Ñ    Females: Males = 3:1

Age

Ñ    Younger patients who have been on renal replacement therapy for longer periods seem to be more predisposed to it

E&M

Ñ    Long-term obesity

Ñ    Recent and sudden weight loss

IS

Ñ    Rapid onset of lesions, which progress rapidly

Ñ    Lesions may be single or multiple

Ñ    Lesions are intensely painful

KUS

Ñ    Chronic renal failure

Ñ    Renal replacement therapy

Ñ    Renal transplantation

Drug History

Trigger factors could be treatment with:

Ñ    Immunosuppressive agents

Ñ    Steroids

Ñ    Infusions of iron dextran

Clinical Features

Examination

IS:

Early lesions:

Ñ    Non-specific violaceous mottling

Ñ    Livedo reticularis

Ñ    Erythematous papules, plaques, nodules

Established lesions:

Ñ    Star-shaped purpura with central necrosis

Ñ    Induration is a feature

Ñ    Excruciatingly tender

Ñ    Multiple lesions may be present and they are arranged along the path of the vasculature

Ñ    Distribution of the lesions may be proximal

Ñ    Distal lesions are more commonly associated with visceral involvement

CVS

Ñ    Intact peripheral pulses:

Helps to differentiate these lesions from lesions associated with peripheral vascular disease

Complications

IS

Ñ    Non-healing ulcers

Ñ    Cutaneous gangrene

Ñ    Sepsis

GIT

Ñ    Gastrointestinal haemorrhage, infarction, organ failure

Investigations

Fluids

Blood

Biochemistry

Ñ    Blood urea and creatinine levels:

Usually high

Ñ    Calcium and phosphate levels:

May be high

Ñ    Calcium-phosphate product:

May be high but calciphylaxis can occur with a normal calcium-phosphate product

Imaging

X-ray

Ñ    Plain films:

Demonstrate vascular calcification within the dermis and subcutaneous tissue

This may be seen in end stage renal failure and is not specific for calciphylaxis

Tissue Diagnosis

Skin biopsy

Ñ    Histology demonstrates calcification within the media of small and medium sized vessels with intimal hyperplasia and fibrosis

Ñ    A mixed inflammatory infiltrate may be seen

Ñ    Subcutaneous calcification and fat necrosis may also be seen

Management

Control

Drugs

Ñ    Phosphate binders:

Reduce phosphate to low normal levels

Ñ    Judicious use of antibiotics

Ñ    Anticoagulation:

If there is associated hypercoagulability

Surgery

Ñ    Wound debridement

Ñ    Parathyroidectomy:

To reduce hypercalcaemia

Lifestyle Adjustments

Ñ    Specific diet:

Address calcium and phosphate homeostasis

Equipment

Ñ    Hyperbaric oxygen:

May be beneficial

Prognosis

Ñ    Not good

Ñ    Mortality rate:

60-80 %

Ñ    Morbidity:

Patients may have to undergo an amputation

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