Urticarial vasculitis

Last Updated: 2025-02-11

Author(s): Anzengruber F., Navarini A.

ICD11: EF40.10

1/20

Mc Duffie, 1973

Urticarial vasculitis, urticarial vasculitis, hypocomplementemic vasculitis, urticaria perstans

  • Up to max. 10% of patients with fixed urticarial skin changes have histological vasculitis
  • Chronic (lasting > 6 weeks), inflammatory vasculitis with recurrent course
  • Clinic characterised by urticarial papules, plaques or angioedema
  • In contrast to urticaria, the lesions persist for more than 24 hours and are localised

  • W : M = 2 : 1
  • Peak of disease usually 5th decade of life
    • Urticarial vasculitis with hypocomplementemia affects almost only women

  • There are two different forms:
    • Urticarial vasculitis with hypocomplementemia (HUVS)
    • Urticarial vasculitis with normocomplementemia

  • Probably due to the formation of immune complexes consisting of IgG antibodies against the collagen-like region of C1q → This is followed by complement activation

 

Frequently associated with:

  • Autoimmune diseases (e.g. systemic lupus erythematosus)

  • Viral infections (e.g. hepatitis B/C)

  • Neoplasms (myeloma, gammopathies, colon carcinoma)

  • Medications (e.g. NSAID, fluoxetine)

Urticular skin changes:

  • partly dolent
  • persistent for a long time (usually 24 - 72 hours)
  • Diascopy: usually purpura in the centre of the wheal
  • Healing often associated with hyperpigmentation
  • Angioedema may also occur

 

Systemic general symptoms:

  • Fever
  • Arthralgias and arthritides in 50% (especially ankle, hand, knee and foot joints)
  • Renal
  • Obstructive lung infestation in 20% (dyspnoea, tussis, haemoptysis)
  • Gastrointestinal symptoms in 20% (abdominal pain, nausea, diarrhoea)
  • Lymphadenopathy

  • Anamnesis
  • Clinic
  • Search for inflammatory focus
  • Laboratory:
    • Elevation of acute phase proteins, half of the patients show hypocomplementemia ( C3 and C4 decreased)
    • Infection serology
    • ANA titre
  • Electrophoresis: Question about gammopathy
  • Direct immunoflourescence: granular immune and complement deposits in the vessel walls of the dermis
  • Skin biopsy: Typical histology
  • Depending on the organ involvement, specific examinations should be performed (if necessary, abdominal sonography, chest X-ray, lung function diagnostics, urine status)

Similar to the picture of leukocytoclastic vasculitis:

  • Leukocytoclastic reaction, vessel wall destruction, nuclear debris, fibrinogen deposits

  • Life-threatening courses are possible

  • Chronic, relapsing course
  • A transition to systemic lupus erythematosus is described in 2/3 of patients

  1. Lubach D (1983) The so-called urticarial vasculitis. Allergologie 6: 300-303
  2. Maurer M, Grabbe J: Urticaria - targeted anamnesis and cause-orientated therapy. Dtsch Arztebl 2008; 105(25): 458-65.
  3. Guha B, Youngberg G, Krishnaswamy G: Urticaria and urticarial vasculitis. Comp Ther 2003; 29: 146-56.
  4. Palazzo E, Bourgeois P, Meyer O, de Bandt M, Kazatchkine M, Kahn MF: Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination. J Rheumatol 1993; 20: 1236-40