Hypocomplementemic Urticarial Vasculitis Syndrome

Hypocomplementemic cutaneous vasculitis is an acquired form of deficiency that leads to chronic decreases in complement components C1, C2, C4, and C3. The syndrome is now called hypocomplementemic urticarial vasculitis syndrome (HUVS) but in the past was identified as SLE-related syndrome, hypocomplementemic cutaneous vasculitis, chronic hypocomplementemic cutaneous vasculitis, or hypocomplementemic vasculitis urticaria syndrome. Patients with this syndrome have several distinct clinical...

C1 Inhibitor Deficiency and Systemic Lupus Erythematosus

Genetic and acquired forms of C1-INH are associated with the syndrome of hereditary angioneurotic edema, which consists of recurrent bouts of noninflammatory swelling involving the subcutaneous tissues, intestinal walls, airways, and lungs. Twice as many males as females are affected. Two major types of the genetic deficiency exist. In approximately 85 of patients, functional and antigenic assays show low serum concentration of the inhibitor. In the variant form, which comprises approximately...