Urticarial vasculitis is a multisystem disease characterized by cutaneous lesions resembling urticaria, except that wheals persist more than 24 h, generally up to 3 to 4 days. Fever, arthralgia, elevated sedimentation rate, and histologic findings of a leukocytoclastic vasculitis are also present. The syndrome is often accompanied by various degrees of extracutaneous involvement. May be cutaneous manifestations of SLE.
Causes of Urticarial Vasculitis
Thought to be an immune complex disease, similar to hypersensitivity vasculitis. Deposition of antigen-antibody complexes in cutaneous blood vessel walls leads to complement activation, resulting in neutrophil chemotaxis; collagenase and elastase released from neutrophils cause vessel wall and cell destruction.
Symptoms of Urticarial Vasculitis
The first symptom of urticarial vasculitis is an urticarial eruption that is often painful or has a burning sensation. In some cases there may be pruritus (itching). Lesions are red patches that may have a white centre and petechiae (small spots of bleeding under the skin) may appear. Lesions usually last for more than 24 hours in a fixed location after which they will slowly resolve spontaneously. Ecchymoses (bruise-like patches) or hyperpigmentation (darkening of the skin) may occur in the healing process.
Diagnosis
Clinical suspicion confirmed by skin biopsy.
Disease Associations SLE and other collagen vascular autoimmune disease.
Treatment
Rule out vascular/connective tissue disease.
First Line H1 and H2 blockers [doxepin (10 mg bid to 25 mg tid) plus cimetidine ( 300 mg tid)/ranitidine (150 mg bid)] plus a nonsteroidal anti-inflammatory agent [indomethacin (75 to 200 mg/d) ibuprofen (1600 to 2400 mg/d)/naprosyn (500 to 1000 mg/d)]
Second Line Colchicine, .6 mg bid or tid or dapsone, 50 to 150 mg/d.
Third Line Prednisone.
Fourth Line Cytotoxic immunosuppressive agents (azathioprine, cyclophosphamide).
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