Angioedema: General Overview

Review Article

Austin J Dermatolog. 2014;1(5): 1022.

Angioedema: General Overview

Cotugno M1, Cánovas-Sanchis S1,2 and Orgaz-Molina J3*

1Department of Internal Medicine, University Hospital Virgen de la Arrixaca, Spain

2School of Nursing, Catholic University San Antonio, Spain

3Burn Unit and Plastic Surgery, University Hospital Virgen de la Arrixaca, Spain

*Corresponding author: Orgaz-Molina J, Burn Unit and Plastic Surgery, University Hospital Virgen de la Arrixaca, El Palmar, ZP: 30120, Murcia, Spain

Received: September 02, 2014; Accepted: September 24, 2014; Published: September 26, 2014


Angioedema diagnosis is usually evident at clinical level. However a precise etiological diagnosis can be more difficult because clinical manifestations are lack of specificity and there are multiple possible causes. A precise diagnosis will not be achieved if the clinician does not have a clear overall view of angioedema. In the present article we try to offer an overview of main features of more important causes of angioedema in order to aid clinicians in their practical activity.

Introduction and Pathophysiological Notes

AE is a rare condition. Hereditary Angioedema (HAE) has an estimated prevalence of 1 in 50.000 to 100.000 of the population [1,2]; the prevalence of Acquired Angioedema (AAE) due to C1-Inh deficiency is unknown, but it has been estimated in 1 for every 10 patients with HAE [3].

Angioedema (AE) is a localized swelling of deep dermis and/or mucosa of the gastrointestinal or respiratory tract. This swelling is due to an increased vascular permeability ("from the Greek "angeio" -vessel- and "oedema" -swelling-). Regarding vascular permeability mastocyte is a main target cell located in skin. Provided with high affinity IgE receptors (FceRI), mastocyte can be stimulated to release different substances with vasoactive potential (such as histamine, proteases, cysteinil leukotrines...) due to binding to sensitized IgE or antibodies generated secondary to autoimmune conditions [4]. On the other hand, bradykinin due to its vasoactive function is a very important molecule, clue in some forms of AE. Factor XIIa produces kallicrein and kallicrein is able to produce bradykinin from high molegular weight kininogens. It is important to know that the molecule responsible of the inhibition of this metabolic chain (both the factor XII and the kallicrein) is C1-Inhibitor (C1-Inh) [4]. Then, it is easily understandable that a C1-Inh deficiency or an increase in activity or amount of kallicrein or factor XII can lead to AE.

Although the syndromic diagnosis is usually easy, the etiological diagnosis can be challenging. A precise diagnosis is important for an adequate management. In this sense, Bork et al described that mortality in patients with HAE who had not been diagnosed was 29% vs 3% in patients who had been diagnosed [5].

Classification of AE

There are different criteria to classify AE. AE can be classified based on the familial history as Hereditary or Acquired (Table 1). When hereditary form is suspected, the next step is to distinguish if it exists a C1-Inh deficiency (HAE I and II) or not (HAE III). Among acquired forms there are several causes; the precise diagnosis depends on medical history. However, the etiology is often not found (idiopathic) [6]. From a therapeutical point of view, it can be more useful a classification based on the pathophysiology as histaminergic and non-histaminergic forms (Table 2), being non-histaminergic forms not associated to urticaria manifestations and usually responsive to antihistamines [4,7].