The dermatology view of hereditary angio-oedema: practical diagnostic and management considerations

Authors


  • Conflict of interest
    M. Maurer has been a paid consultant, has received unrestricted or educational grants and has received funding to support the work on this project; L.C. Parish has been a paid consultant, has received unrestricted or educational grants, has received funding to support the work on this project and has received a grant for participating in the writing of a manuscript on this subject.

  • Funding source
    None.

M. Maurer. E-mail:marcus.maurer@charite.de

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.

Ancillary