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Key Features

  • Essential (mixed) cryoglobulinemia is a vasculitis associated with cold-precipitable immunoglobulins (cryoglobulins)

  • Causes

    • Hepatitis C infection (usual)

    • Other overt or occult infections

    • Viral, bacterial, and fungal infections

    • Some connective tissue diseases

Clinical Findings

  • Purpuric and necrotizing skin lesions in dependent areas

  • Arthralgias

  • Fever

  • Hepatosplenomegaly

Diagnosis

  • Serum complement levels are low

  • Rheumatoid factor often elevated

  • Kidney biopsy: crescent formation, glomerular capillary thrombi, membranoproliferative glomerulonephritis (MPGN)

Treatment

  • Aggressive targeting of the causative infection

  • Pulse corticosteroids, plasma exchange, rituximab, and cytotoxic agents have been used when

    • There is little risk of exacerbating the underlying infection

    • No infection is present

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