Clinical Features

  • Consists of 2 key elements:
    1. Clinical manifestations of urticaria
      • Skin lesions differ from common urticaria
        • Wheals may have a central dark or red appearance
        • Lesions last >24 h
          • Note that large urticarial plaques may need more than a day to resolve and this should not be confused with vasculitis lesions
        • Often resolve with residual hyperpigmentation
        • Typically painful with a burning/stinging sensation
        • Pruritis less common than true urticaria
        • In contrast to above, a series of UV patients showed 60% with lesions lasting <24 h, 80% with pruritic lesions, and 8% with painful/tender lesions
    2. Histology demonstrating leukocytoclastic vasculitis (largely involving postcapillary venules)
      • 80% will have positive immunofluorescence for immunoglobulin, complement, or fibrin (not specific for UV and can be seen in SLE)
  • Often presents with other systemic symptoms:
    • Musculoskeletal (arthritis)
    • Pulmonary (COPD)
    • Renal
    • GI
  • Clinical subtypes may be differentiated by complement levels:
    • CH50, C3, C4, C1q
    • To conclusively state as normal should be repeated 2-3 times over several months during both active disease and quiescence

Normocomplementemic UV (NUV)
Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS)------
Hypocomplementemic UV (HUV)
  • Most cases are idiopathic
  • Can be secondary to:
  • Diagnostic criteria - if criteria not met, considered to have HUV
    • Major (need both)
      • Urticaria >6 months
      • Hypocomplementemia
    • Minor (2 or more)
      • Venulitis of the dermis (established via biopsy)
      • Positive C1q precipitin test with an associated suppressed C1q level------
      • Arthralgia or arthritis
      • Mild glomerulonephritis
      • Uveitis or episcleritis
      • Recurrent abdominal pain

  • Clinical Features
    • Urticarial vasculitis is the dominant feature
    • Angioedema (can be the presenting feature) - 50%
    • COPD - 50%
    • Ocular inflammation (uveitis) - 30%
    • Can get glomerulonephritis
    • Many features resemble SLE (some propose it is a subset of SLE)

  • Lab findings
    • C3, C4 can be undetectable to low normal
    • C1q low in all patients when disease is active
    • Anti-C1q antibodies (C1q precipitins) detectable in all patients
    • Anti-dsDNA and anti-Sm are uncommon
    • ANA frequently positive
  • Have low complement and UV but otherwise do not meet criteria for HUVS
  • Most cases due to:
    • SLE
    • Sjogren's syndrome------
    • Serum sickness
    • Neoplasia

UV clinical sxs.png
UV dx signs.png

Other Conditions Associated with UV

  • Schnitzler's syndrome — monoclonal IgM or IgG gammopathy with intermittent spiking fever, weight loss, bone pain, arthralgias, lymphadenopathy, and nonpruritic urticaria (later may become pruritic); may be due to circulating immune complexes and complement activation, responds to anakinra (IL1R antagonist)Schnitzler criteria.png
    Cinical biologic findings of Shnitzler sd.png
  • Cryopyrin-associated autoinflammatory syndromes (CAPS)
  • Cryoglobulinemia, often associated with Hepatitis C



Evaluation of Urticarial Vasculitis (Langford)

  • Obtain in patients with compatible clinical and histologic presentation
  • Complement levels - to conclusively state as normal should be repeated 2-3 times over several months during both active disease and quiescence
    • CH50, C3, C4
    • C1q
    • Anti-C1q antibodies (C1q precipitins) - found in 100% of patients with HUVS
      • May also be found in Felty's syndrome, SLE, Sjogren's, MPGN
  • CBC with differential
  • Urinalysis
  • Chemistries
  • Hepatitis B and C serologies
  • ANA, anti-dsDNA, anti-Ro (SS-A), anti-La (SS-B), anti-Smith, anti-RNP, anti-Scl70, anti-Jo
  • ANCA
  • RF and anti-CCP antibody
  • SPEP
  • Cryoglobulins
  • Other investigations as appropriate:
    • Chest XR, CT
    • Skeletal XR in patients with joint pain
    • PFTs (to look for COPD)

Treatment

  • Referral to rheumatologist
  • Treat underlying disease if present
  • For cutaneous lesions: prednisone, hydroxychloroquine, dapsone, colchicine
  • For severe systemic disease: systemic immunosuppression may be required
  • For HUVS: as above, some series have shown stabilization with cyclosporine


References