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Vasculitis (Leukocytoclastic Vasculitis)

A broad spectrum of disorders, vasculitis is characterized by inflammation and necrosis of blood vessels. Its clinical effects depend on the vessels involved and reflect tissue ischemia caused by blood flow obstruction.

The prognosis is also variable. For example, hypersensitivity vasculitis is usually a benign disorder limited to the skin, but more extensive polyarteritis nodosa can be rapidly fatal.

Vasculitis can occur at any age, except for mucocutaneous lymph node syndrome, which occurs only during childhood. Vasculitis may be a primary disorder or secondary to other disorders, such as rheumatoid arthritis or systemic lupus erythematosus.

Causes

Often, doctors don't know what triggers vasculitis. However, it appears that, in some people, the immune system mistakes the blood vessels for foreign substances and attacks them. The immune system sends chemicals and immune cells, called antibodies, to fight the invader. These damage the blood vessels, which leak and cause swelling. In some cases, vasculitis appears to be triggered by an allergic response to a specific substance, such as a drug. 

Signs and symptoms

• skin lesions usually located on legs, buttocks, or trunk
• palpable (able to be felt with the fingers) purpura
  • multiple papules (small, solid, raised skin areas)
  • purpura ( bleeding into the skin )
• blisters on the skin
• urticaria (hives), may last longer than 24 hours
• necrotic ulcers (open sores with dead tissue)

Diagnosis

Diagnosis of any type of vasculitis involves demonstrating the presence of a strong inflammatory process. Tests which reveal inflammation throughout the body include erythrocyte sedimentation rate , blood tests which may reveal anemia and increased white blood cells, and tests to demonstrate the presence of immune complexes and/or antibodies circulating in the blood. An x-ray procedure, called angiography , involves injecting dye into a major artery, and then taking x-ray pictures to examine the blood vessels, in order to demonstrate the presence of inflammation of the vessel walls. Tissue samples (biopsies) may be taken from affected organs to demonstrate inflammation.

Treatment

The aim of treatment is to minimize irreversible tissue damage associated with ischemia. In secondary vasculitis, treatment focuses on the underlying disorder. Primary vasculitis is mainly treated with drugs.

Treatment may involve removal of an offending antigen or use of anti-inflammatory or immunosuppressive drugs. For example, antigenic drugs, food, and other environmental substances should be identified and eliminated if possible.

Drug therapy in primary vasculitis frequently involves low-dose cyclophosphamide (2 mg/kg by mouth daily) with daily corticosteroids. In rapidly fulminant vasculitis, cyclophosphamide dosage may be increased to 4 mg/kg daily for the first 2 to 3 days, followed by the regular dose. Prednisone should be given in a dose of 1 mg/kg daily in divided doses for 7 to 10 days, with consolidation to a single morning dose by 2 to 3 weeks.

When the vasculitis appears to be in remission or when prescribed cytotoxic drugs take full effect, corticosteroids are tapered down to a single daily dose and then to an alternate-day schedule for 1 to 2 months, after which steroids are slowly discontinued.

Prevention

Avoid exposure to medications to which there are known allergies.