Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis.
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.
Farkas
H
H
Allergology and Angioedema Outpatient Clinic, Semmelweis University, Kútvölgyi Centre, Budapest, Hungary. farkash@kut.sote.hu
Szongoth
M
M
Bély
M
M
Varga
L
L
Fekete
B
B
Karádi
I
I
Füst
G
G
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*
D000962:Antimalarials; D003174:Complement C1 Inactivator Proteins; D006886:Hydroxychloroquine; D002738:Chloroquine; D003165:Complement System Proteins