Chronic angioedema. Three relevant cases.
Three cases of clinical angioedema are reported in which the etiopathogenetic involvement of qualitative and quantitative complement disorders was demonstrated. The first patient had a functional deficit in C1 inhibitor, the second had a decrease in CH50, and the third, a reduction in the C1q, C3, and C4 fractions. The cases are interesting because of occasional difficulties in the causal diagnosis, the severity of the symptoms, which can include laryngeal edema, and, finally, the favorable outcome achieved with correct medication, which did not include antihistamines or steroids. The clinical picture of hereditary angioedema is characterized by the familial occurrence of the process, although this apparently was absent in these cases. Two patients experienced laryngeal edema and none had abdominal manifestations. The treatment of choice for angioedema of these characteristics is antifibrinolytic agents, which achieve good results in 70% of the patients. Epsilon-aminocaproic acid and tranexamic acid inhibit the formation of plasmin and fragments of the Hageman factor, thus inhibiting kallikrein and bradykinin production. The androgens danazol and stanazolol have been used since the 1970s, and stanazolol proved to be very effective in two of our patients.
Sánchez Palacios
A
A
Allergy Unity, Hospital Insular, Las Palmas de Gran Canaria, Spain.
Schamann Medina
F
F
García Marrero
J A
JA
D000328:Adult; D015119:Aminocaproic Acid / Q000627:therapeutic use; D000799:Angioedema / Q000188:drug therapy / Q000209:etiology* / Q000235:genetics; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000235:genetics / Q000627:therapeutic use; D015922:Complement C1q / Q000172:deficiency; D003176:Complement C3 / Q000172:deficiency; D003181:Complement C4 / Q000172:deficiency; D003165:Complement System Proteins / Q000172:deficiency* / Q000235:genetics; D003613:Danazol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007819:Laryngeal Edema / Q000188:drug therapy / Q000209:etiology; D008297:Male; D008875:Middle Aged; D013197:Stanozolol / Q000627:therapeutic use; D014148:Tranexamic Acid / Q000627:therapeutic use
D000933:Antifibrinolytic Agents; D003174:Complement C1 Inactivator Proteins; D003176:Complement C3; D003181:Complement C4; D013197:Stanozolol; D014148:Tranexamic Acid; D015922:Complement C1q; D003165:Complement System Proteins; D003613:Danazol; D015119:Aminocaproic Acid