Background:

Hereditary angioedema is classified as a primary immunodeficiency of the complement system because it is characterized by the absence of C1 esterase inhibitor (C1-INH) and by the periodic edema of any region of the body that involves soft tissue.

Objective:

To characterize the adult patients with a confirmed diagnosis of hereditary angioedema who have been treated in the Clinical Allergology Department of Hospital México de la Caja Costarricense del Seguro Social.

Methods:

The study was retrospective and observational. The information was obtained from the clinical records of the patients with a confirmed diagnosis of hereditary angioedema that were being controlled in the Allergology Department of the “Hospital México de la Caja Costarricense del Seguro Social”.

Results:

A total of 14 patients; seven men and seven women, were identified. The average was of 36.6 years of age. The most frequent clinical manifestations were peripheral edema and abdominal pain. Only three patients presented laryngeal edema at some point in their evolution. 12 cases corresponded to hereditary angioedema type I.

Conclusion:

The clinical characteristics of all the documented cases corresponded to those described for this pathology, although only a few patients had a history of severe manifestations.