Content » Vol 81, Issue 4

Clinical Report

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
DOI: 10.1080/00015550152572985


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.


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