Actas dermo-sifiliográficas
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C1 inhibitor deficiency is a rare syndrome clinically characterized by recurrent episodes of swelling of subcutaneous tissue or angioedema. It can involve the skin, upper respiratory airways and abdomen. There are two main types: hereditary and acquired. ⋯ Diagnosis of this rare syndrome is based on clinical features and characteristic alterations of laboratory tests. The acute attack should be treated as quickly as possible. Prophylactic therapy is indicated in certain circumstances (dental procedures, oral surgery).