Acquired angioedema due to C1-inhibitor deficiency is a very rare disorder that usually
appears in patients with lymphoproliferative and/or autoimmune diseases. This type
of swelling is bradykinin mediated and does not respond to antihistamines, corticosteroids,
or epinephrine. The symptoms usually appear in patients older than 40 years with recurrent
episodes of angioedema without wheals. The family history is negative. The swelling
could affect any tissue, but most frequently is located at the face, lips, tongue,
larynx, or extremities. In the gastrointestinal tract, it causes pain, nausea, vomiting,
and diarrhea. The upper respiratory airway oedema is a potentially lift-threatening
condition due to asphyxiation. The oedema attacks may precede the symptoms of the
causative disease for months or years. In most cases, the treatment of the underlying
disease resolves the angioedema episodes. Here we report a case of C1-INH-AAE caused
by non-Hodgkin lymphoma in a patient diagnosed many years before with myasthenia gravis
whose angioedema symptoms resolved after the specific treatment of lymphoma.
