Hereditary angioedema in oral surgery: overview of the clinical picture and report of a case.

p p t ngioedema is a pathologic condition first described y Quincke and Osler. It can be genetically deterined or acquired, and it is caused by a vascular eaction induced by deficiency or functional altertion of the C1 inhibitor (C1-INH), an enzyme inolved in the regulation of complement, contact, firinolytic, and coagulation systems. Two forms of angioedema have been described in he literature: hereditary angioedema (HAE) and acuired or idiopathic angioedema (AAE). HAE is characterized by dominant autosomal transission, early onset, and a positive family history. It s caused by a mutation of the gene for C1-INH, ocalized on chromosome 11. Type 1 HAE is the most requent form (85% of cases) and is associated with eficiency of functional C1-INH in plasma (10%-30% f normal values). Type 2 HAE is characterized by ormal or elevated plasma levels of dysfunctional C1NH. Type 3 HAE occurs mainly in women. Affected ersons display the typical clinical features of C1-INH