Symptoms

• Athralgia, purpura, skin ulcers, glomerulonephritis, peripheral neuropathy

Classification

• Type 1 - monoclonal Igs

• Type 2 - monoclonal IgM (or IgG or IgA) with RF

• Type 3 - Mixeture of IgG and IgM

Incidence

• 1 in 100k

Pathogenesis

• Chronic immune stimulant and/or lymphoproliferation

• Enhanced immune complex formation

Clinical

• Type 1

  • Digital ischaemia, livedo reticularis, skin necrosis

  • Skin changes in 80%

  • Raynaud Phenomenon

  • Peripheral neuropathy in up to 45%

  • Hyperviscosity symptoms including blurred vision, headache, vertigo, nystagmus, dizziness, deafness, diplopia, ataxia, confusion