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Rev Alerg Mex. 2017 Oct-Dec;64(4):493-498.

[Hereditary angioedema by C1 inhibitor-deficit: Diagnostic and therapeutic challenges. Case report].

[Article in Spanish; Abstract available in Spanish from the publisher]

Author information

1
Centro de Neumología y Alergias, San Pedro Sula, Cortés, Honduras. alvaro_mayorga@yahoo.com.

Abstract

in English, Spanish

BACKGROUND:

Hereditary angioedema is a disease which manifests itself with episodes of spontaneous edema on skin, mucosal and airway. Treatment includes acute and prophylactic approach to minimize the attacks and severity. In many parts of the world, androgen derivatives, antifibrinolytic and fresh frozen plasma are the therapies available for prophylaxis.

CLINICAL REPORT:

16 years old teenager of without history of immune decease, has in the course of 1-year repetitive episodes of painless, non-pruritic angioedema, does not respond to antihistamine therapy, corticosteroids or adrenaline; fresh frozen plasma is applied in 1 occasion exacerbating episode with severity. The diagnosis is delayed because of the unavailability of the study in the country, so it is shipped abroad confirming the deficit of C1 Inhibitor (7.1 μg/mL). Initiating prophylactic therapy with Danazol, with subsequent episodes decreased.

CONCLUSION:

The delay diagnosis involves considerable risk in these patients; the importance of long-term prophylactic treatment is ratified in the use of androgens, being as an available option in developing countries.

KEYWORDS:

Angioedema; Deficit C1 inhibitor; Hereditary angioedema

PMID:
29249111
DOI:
10.29262/ram.v64i4.310
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