BACKGROUND:

Hereditary angioedema is a rare disease characterized by recurrent episodes of edema anywhere in the body, especially in the extremities, face and airways. There are three types of the disease depending on its cause, the most infrequent being III with normal C1 inhibitor level and function. Its pathophysiology is poorly understood; therefore, its diagnosis is difficult. Its treatment has advanced in recent years, although much remains to be defined, especially during pregnancy.

OBJECTIVE:

To evaluate the available literature related to hereditary angioedema and its medical care in pregnant women.

CLINICAL CASE:

30-year-old female patient, during her first pregnancy. The only personal history of note was hereditary angioedema type III, diagnosed 10 years earlier after several episodes of orofacial angioedema. Throughout the pregnancy, several crises of the disease occurred, requiring treatment in acute episodes and maintenance treatment in the third trimester. Finally, one delivery was instrumented by vaccum, due to risk of loss of fetal well-being with good maternal and fetal outcome in the immediate postpartum period.

CONCLUSIONS:

Hereditary angioedema type III is a very rare and little-known disease that requires establishing a protocol and standardization of clinical care, especially in pregnant women, which will help to provide quality information and assistance to these patients.