HYPOCOMPLEMENTEMIC URTICARIAL VASCULITIS SYNDROME IN IDENTICAL-TWINS

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a syndrome of recurrent urticarial vasculitis, arthralgia/arthritis, and hypocom plementemia. Angioedema, ocular inflammation, glomerulonephritis, and obstructive lung disease are other clinical findings. Although the eti ology of HUVS is unknown, its resemblance to systemic lupus erythemato sus (SLE) suggests a similar pathogenesis. SLE is known to occur in id entical twins. This is the first report of a pair of identical twins w ith HUVS. Concordance for HUVS in identical twins suggests that the pa thogenesis of the disease involves abnormal genetic immunoregulation.