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Dermatology. 2015;230(4):324-31. doi: 10.1159/000371814. Epub 2015 Feb 20.

Distinct conditions support a novel classification for bradykinin-mediated angio-oedema.

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1
Department of Dermatology, Angers Hospital, L'UNAM University, Angers, France.

Abstract

BACKGROUND:

Angio-oedema (AO) can be attributable to bradykinin (BK) accumulation, as is the case for prototypical hereditary AO (HAO) due to C1 inhibitor (C1-INH) deficiency. However, our clinical experience in a reference centre has shown that some patients display a clinical history suggestive of HAO, but exhibit normal C1-INH function, have no mutation in the causative genes associated with HAO (SERPING1, F12), and report no intake of drugs known to promote AO.

OBJECTIVE:

We sought to determine the frequency and distribution of different AO subtypes suspected to be BK-mediated AO (BK-AO) and defined by clinical, history and biological criteria (enzyme activities implicated in BK formation and catabolism).

METHODS:

The files of all patients referred to our centre for suspected BK-AO were retrospectively analysed.

RESULTS:

The distribution of patients (n = 162) was 16 and 4% with a hereditary deficiency of C1-INH or a gain of factor XII function, respectively, 29% with iatrogenic BK-AO, 21% with non-iatrogenic defective kininase activity and 30% with idiopathic increased kinin formation.

CONCLUSION:

BK-AO may be caused by multiple inherited or acquired factors triggering BK accumulation. Therefore, we propose a novel typology for BK-AO based on the imbalance of production/catabolism of BK.

PMID:
25720836
DOI:
10.1159/000371814
[Indexed for MEDLINE]

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