Angiooedema due to acquired deficiency of C1-esterase inhibitor associatedwith leucocytoclastic vasculitis

A hereditary and an acquired type of CI-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurre nt subcutaneous and submucosal angiooedema. Acquired CI-esterase inhibitor deficiency has been observed in association with lymphoproliferative disord ers, malignancy, autoimmune diseases and infections. We report on a case wi th the acquired form of the disease accompanied by leucocytociastic vasculi tis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, CI-esterase inhibitor concentration and ac tivity as well as C1 levels, all returned to normal.