Angioedema without urticaria: a large clinical survey

Abstract

A ngioedema -a self-limited, localized swelling that involves subcutaneous tissues or mucosa -can involve the face and other areas. Skin colour is usually unchanged, although it may be heralded by erythema marginatum. There is no burning sensation and itchiness is generally absent. Lesions tend to be large and without clear demarcation; most resolve within 2 or 3 days. 1Frequently the result of the release of histamine, angioedema is often associated with urticarial flares (hives; the association is often called urticaria-angioedema syndrome) and responds to antihistamines and corticosteroids.2 Occasionally, angioedema occurs without urticaria, does not respond to drugs against allergies and tends to last 5-7 days -for example, when it is caused by a deficiency in plasma of C1 esterase inhibitor (C1Inh).To investigate and form classifications for different forms of angioedema unaccompanied by urticaria, we analyzed 929 consecutive patients referred to our university hospital over an 11-year period (January 1993 through December 2003). MethodsOur outpatient clinic for angioedema is a tertiary-level centre where patients are referred mostly by specialists. All clinic patients with a history of recurrent angioedema without major urticaria between January 1993 and December 2003 were considered for the study.From each patient, we obtained a clinical history with information about allergies and their relation to potential causative agents (e.g., foods, drugs, chemicals) and carried out a complete physical examination. We obtained sinus and dental radiographs as well as test results for blood cell count, serum protein electrophoresis, erythrocyte sedimentation rate, Creactive protein, hepatic enzymes, renal function, thyroid function and anti-tissue antibodies (antinuclear antibodies and antibodies to extractable nuclear antigens, to doublestranded DNA, to thyroglobulin and to thyroperoxidase). Stool examinations (for ova and parasites) and urinanalyses were carried out, and pharyngeal and urine samples were cultured. Further specific analyses were performed if deemed necessary.If all test results were negative, response to long-term H1-

Presentation, diagnosis and treatment of angioedema without wheals: a retrospective analysis of a cohort of 1058 patients

“…In 2006, we reported the results of a large clinical survey initiated in 1993 of patients with angioedema [14]. In the present study, we have reevaluated and updated this cohort, considering knowledge acquired in the last few years with regard to diagnosing and treating angioedema.…”

New topics in bradykinin research

“…Sporadic angioedema that is unresponsive to high-dose antihistamines and is not linked to C1-INH deficiency (i.e. acquired nh-IAE) is clinically reminiscent of bradykinin-mediated angioedema (65). Such patients show markedly increased bradykinin levels at the site of oedema, which is further proof of a link between angioedema development and bradykinin (66).…”

Drug-induced angioedema: an update on new cases and new drugs

“…Angioedema refers to a group of disorders with multiple etiology, similar clinical manifestations and different mechanisms such as genetic mutations, allergic reaction and non-allergic reactions (29,30). Hereditary angioedema, due to a genetic deficiency of C1 inhibitor is the best known, but there is a rare form of angioedema, caused by increased levels of circulating bradykinin (31,32).…”