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ABSTRACT
Introduction: Hereditary angioedema is an autosomal dominant genetic disease, associated with increased levels of bradykinin. It is classified into 3 types according to the C1-INH enzyme. The diagnosis is clinical and laboratory. Its treatment is divided into short- and long-term and crisis prophylaxis.
Case report: 40-year-old female patient who came to the emergency service for labial edema without resolution with corticosteroids. The tests for IgE, C4 and C1 esterase inhibitors had a low result. She currently uses danazol prophylactically and fresh frozen plasma in crises.
Discussion: HAE is one of the rarest causes of all types of angioedema. Most cases occur spontaneously in predisposed patients. Diagnostic criteria for HAE with C1-INH deficiency were established. Many professionals are unaware of HAE, which ends up being underdiagnosed and undertreated.
References
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