Purpose: Nonallergic isolated angioedema is an uncommon clinical syndrome raising difficult diagnosis and therapeutic problems. Occurrences linked to a C1Inh are the predominant ones and have to be examined as a priority, taking into account the specificity of the associated follow-up.
Current knowledge and key points: Diseases with a clinical profile close to hereditary angioneurotic edema, but without C1Inh anomaly, have been described recently. It is in fact family cases, concerning only women, where estrogens seem to play a dominant role. Angioedema's secondary aspects are gathering various pathologies (vasculitis, Gleich's syndrome, angioedema initiated by physical agents). The role played by some drugs must not be forgotten, mainly angiotensin converting enzyme inhibitors, which are at the origin of angiodema in nearly 0.5% of users.
Future prospect and projects: Uncontrolled activation of the contact system seems to play a major role in the main part of these angiodemas. The efficiency of the tranexaminic acid (which modulates its activation) is to be taken as evident. The key to the future seems to be the development of plasmin and bradykinin inhibitors.