title: Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
abstract: 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.
authors.affiliation: Allergology and Angioedema Outpatient Clinic, Semmelweis University, Kútvölgyi Centre, Budapest, Hungary. farkash@kut.sote.hu
authors.forename: H
authors.initials: H
authors.lastname: Farkas
authors.forename: M
authors.initials: M
authors.lastname: Szongoth
authors.forename: M
authors.initials: M
authors.lastname: Bély
authors.forename: L
authors.initials: L
authors.lastname: Varga
authors.forename: B
authors.initials: B
authors.lastname: Fekete
authors.forename: I
authors.initials: I
authors.lastname: Karádi
authors.forename: G
authors.initials: G
authors.lastname: Füst
mesh_terms: D000328:Adult; D000799:Angioedema / Q000188:drug therapy / Q000201:enzymology*; D000962:Antimalarials / Q000627:therapeutic use; D002738:Chloroquine / Q000627:therapeutic use; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D003165:Complement System Proteins; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D016896:Treatment Outcome; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000188:drug therapy / Q000201:enzymology*
chemical_list: D000962:Antimalarials; D003174:Complement C1 Inactivator Proteins; D006886:Hydroxychloroquine; D002738:Chloroquine; D003165:Complement System Proteins