[Acquired angioedema]

Abstract

Acquired angioedema are rare. They are associated with monoclonal gammapathies of uncertain significance (MGUS) or lymphomas. They give the same symptoms as the hereditary form and the same laryngeal risk. They are characterized by a low level of C4, C1Inh and C1q. They are linked to the consumption of C1Inh by the lymphoid cells or to the presence of anti-C1Inh autoantibodies. They must be treated by symptomatic treatment when attack occur (C1Inh concentrate and icatibant). The use of rituximab needs to prove its efficiency.