Recurrent angioedema in childhood: hereditary angioedema or histaminergic angioedema?

Background: Recurrent angioedema is a rare entity during childhood. This study aimed to clarify differences between hereditary angioedema (HAE) and histaminergic angioedema (HA) in children.

Methods: Fifty-seven children with HAE (male 36.8%, 8.9 years [5.4-12.5]) and 42 children with recurrent HA (male 42.9%, 11.5 years [8.1-16.8]) were analyzed.

Results: The median age at symptom onset (6 [3-10]; 7.8 [4.5-13] years), frequency of angioedema episodes within last year (3 [2-5]; 5 [2-10]), and duration of symptoms (48 [24-48]; 24 [12-48] hours) were similar in the HAE and HA group, respectively. Recurrent urticaria was observed in 7.3% (n = 3) of patients in the HAE group and in 45.2% (n = 19) of the HA group (P < .001). While angioedema episodes involving the lips (n = 30; 71.4%; P = .035) and eyelids (n = 28; 66.7%; P = .012) were observed more frequently in the HA group, gastrointestinal involvement/abdominal pain (n = 15; 36.6%) was more common in HAE (P < .001). Itching as a prodromal symptom was detected in 47.6% (n = 20) of HA patients versus 14.6% (n = 6) of those with HAE (P = .002). In the logistic regression analysis for the diagnosis of HAE, a family history of angioedema (OR = 58.289 [95% CI 10.656-318.853], P < 001) and trauma (OR = 35.208 [95% CI [4.368-283.794]], P = .001) as a triggering factor were determined to be independent variables.

Conclusion: A family history of angioedema, trauma as a triggering factor, and abdominal pain should suggest the diagnosis of HAE and the need for further investigation.