Urticarial vasculitis

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

• Depending on the severity of the disease

• Symptomatic therapy: antihistamines, often not sufficient: e.g: Levocetirizine

• Use of glucocorticoids such as prednisone, especially in the hypocomplementemic variant: 

  • Start with prednisolone 60-100 mg/day. Reduction according to the clinic

  • Maintenance dose should be below the Cushing's threshold

  • If therapy is not successful: combination with azathioprine 50-100 mg/day p.o. or mucophenolate mofetil (2.0 g/day p.o.)

• Multiple other options including IL-1 blocking agents

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