title: The dermatology view of hereditary angio-oedema: practical diagnostic and management considerations.
abstract: Hereditary angio-oedema (HAE) is characterized by recurrent, localized, non-pitting, non-pruritic, non-urticarial oedema. Nearly all patients experience skin swelling as a feature of HAE. There may be painful abdominal attacks, accompanied by nausea and vomiting. The disease is life-threatening should laryngeal oedema occur. HAE results from a deficiency or dysfunction of C1 inhibitor, a plasma protein with an important role in regulating the contact, complement and fibrinolytic systems. Effective management of HAE should include a plan for treatment of attacks, as well as routine and preprocedure prevention. Acute and prophylactic therapy with C1 inhibitor therapy for correcting the underlying deficiency in HAE is a valuable option.
authors.affiliation: Department of Dermatology, Venereology and Allergy, Charité - University Medicine, Berlin, Germany. marcus.maurer@charite.de
authors.forename: M
authors.initials: M
authors.lastname: Maurer
authors.forename: L C
authors.initials: LC
authors.lastname: Parish
mesh_terms: D054179:Angioedemas, Hereditary / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics / Q000517:prevention & control; D003172:Complement C1 / Q000037:antagonists & inhibitors*; D003174:Complement C1 Inactivator Proteins / Q000627:therapeutic use; D006801:Humans; D009154:Mutation
chemical_list: D003172:Complement C1; D003174:Complement C1 Inactivator Proteins