URTICARIAL VASCULITIS

By Gary M. White, MD

Urticarial vasculitis This patient had urticarial plaques that left annular purpuric rings.


Urticarial vasculitis (UV) may be defined as urticarial lesions that histologically show vasculitis. In contrast to classic urticaria, UV lesions persist at the same location for more than 24 hours.

Urticarial vasculitis is not a specific disease but instead a clinical finding. A search for a cause of the vasculitis should be undertaken. The most common associated condition is systemic lupus erythematosus. Other possibilities include Sjögren's syndrome, serum sickness, viral infections (e.g. hepatitis C, infectious mononucleosis), mixed cryoglobulins, a monoclonal IgM gammopathy (e.g. Schnitzler syndrome), IgA multiple myeloma, fluoxetine administration, and rarely neoplasm. Hypocomplementemia is common and when it does occur, the association with SLE is highly likely. In one case, the lesions of urticarial vasculitis were induced by cold exposure and an IgG was found on serum protein electrophoresis (SPEP).

Autoantibodies to vascular endothelial cells were found in 82% of patients with urticarial vasculitis and SLE, 70% of patients with hypocomplementemic urticarial vasculitis, and in 14% of patients with urticarial vasculitis alone. They were found in only 32% of patients with SLE without urticarial vasculitis.

Complement Levels

Urticarial vasculitis patients can be divided into those with normal complement levels (normocomplementemic) and those with depressed complement levels (hypocomplementemic). Normocomplementemic urticarial vasculitis tends to have a more benign course, whereas hypocomplementemic urticarial vasculitis is often associated with systemic disease and a more complicated disease course.

Clinical

Urticarial lesions lasting more than 24 hours at any given location are characteristic. Pain and burning are more common than with typical urticaria. Diascopy may blanch much of the erythema, but hyperpigmentation and/or purpura remain. When the urticarial changes resolve, often purpura or other color change persists for a time. Angioedema may be associated.

Systemic Findings

Systemic findings have included anemia, arthralgias, abdominal pain, glomerulonephritis, ocular inflammation, and pulmonary disease. Laboratory abnormalities may include elevated erythrocyte sedimentation rate (ESR), decreased complement levels, decreased or absent C1q, and C1q autoantibody. In one study, the C1q autoantibody was found in 100% of patients with hypocomplementemic urticarial vasculitis syndrome (HUVS), 35% of patients with SLE, and almost no controls.

Workup

Workup may include drug history (e.g. dexfenfluramine), skin biopsy, CBC, ANA, Ro, La, rheumatoid factor, ESR, urine analysis, CH50, C3,C4, liver enzymes, hepatitis C serology, cryoglobulins, and serum protein electrophoresis (SPEP).

Treatment

If a specific cause is found, it should be treated directly. Otherwise, prednisone may be used (e.g. 40-–60 mg/day initially, then tapered). Azathioprine has been used (e.g. 50–-100 mg/day). Other steroid-sparing agents include dapsone and hydroxychloroquine.

Agents to try if these fail include indomethacin and colchicine. Urticarial vasculitis caused by hepatitis C has responded to interferon-therapy.

Additional Pictures

Note the purpura left when the urticarial lesion fades.
Urticarial vasculitis showing residual purpura

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.