clinical features

Last reviewed 10/2021

Classically the symptoms begin during childhood and continue throughout the adult life. Symptoms may become worse around puberty (1).

The main clinical feature is the intermittent accumulation of oedema fluid in the skin or mucus membranes. Also abdominal pain without utricaria may occur(2). . The most commonly affected areas are:

• face
• tongue
• arms
• legs
• hands
• feet
• larynx
• gastrointestinal tract

A prodrome of tingling sensation may accompany many attacks but in around one third of the cases attacks are preceded by erythema marginatum, a nonpruritic, serpiginous rash (1).

The oedema is not pruritic and lasts 24-72 hours (1).

Gastrointestinal oedema often causes colicky abdominal pain, nauseas and vomiting (1).

Oedema of the larynx and pharynx is a life threatening manifestation of hereditary angioedema due to the risk of death by asphyxiation (1).

Reference:

• 1. Zuraw B.L. Hereditary angioedema. NEJM 2008: 359:1027-1036
• 2. Gompels M.M et al. C1 inhibitor deficiency: consensus document. Clin Exp Immunol. 2005; 139(3): 379-394.