Angiooedema Due to Acquired Deficiency of C1-Esterase Inhibitor Associated with Leucocytoclastic Vasculitis
Henriette Farkas A1, Mariann Szongoth A2, Miklós Bély A3, Lilian Varga A4, Béla Fekete A5, István Karádi A5, George Füst A5
A hereditary and an acquired type of C1-esterase inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-esterase inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-esterase inhibitor concentration and activity, as well as C1 levels, all returned to normal.