LIFE-THREATENING ANGIONEUROTIC-EDEMA CAUS ED BY ACQUIRED C1-INHIBITORDEFICIENCY ASSOCIATED WITH PARAPROTEINEMIA AND LIVEDO-RETICULARIS

A 61-year-old patient with life-threatening angioneurotic oedema was f ound to have an acquired C1-inhibitor (C1-INH) deficiency. In addition to lowered serum levels of C1-INH (both protein concentration and enz ymatic activity), C2, C4 and CH50, which are characteristic for the he reditary form of angioneurotic oedema, markedly lowered Clq was found, which is typical for the acquired form. There were no antibodies agai nst C1-INH. Repeated thorough examination disclosed no neoplasm, thoug h the presence of neoplasm has often been reported to be associated wi th the acquired C1-INH deficiency. However, the patient showed persist ent paraproteinaemia and paraproteinuria and developed livedo reticula ris. Treatment with danazol resulted in a rise of the complement fract ion levels and cessation of angioneurotic oedema. Paraproteinaemia and livedo reticularis persisted unchanged.