An ABC of the Warning Signs of Hereditary Angioedema.

Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased bradykinin (BK). This means that special therapies are needed for attacks that do not respond to traditional antiallergic therapies involving antihistamines, corticosteroids, and epinephrine. The recurring attacks may disable patients and lead to frequent visits to emergency rooms where misdiagnoses are common. HAE attacks may be fatal when upper-airway edema occurs, if proper treatment with a C1 inhibitor concentrate or BK receptor antagonist is not administered or an emergency tracheostomy is not performed. We propose a mnemonic method for the warning signs of HAE for the use as a diagnostic tool, i.e., the so-called "ABC" of the warning signs of HAE. The letters represent the following: A = Angioedema, B = Bradykinin, C = C1 inhibitor, D = Distress factors, E = Epinephrine nonresponsive, F = Family history, and G = Glottis/Gastrointestinal edema. To avoid fatalities, medical staff and patients, including family members, must be aware of HAE. An alphabetical mnemonic method has been developed and we hope it may benefit patients.

Clinical characteristics of patients with mustard hypersensitivity.

BACKGROUND: Although mustard is frequently consumed in Spain and elsewhere, only isolated case reports of mustard allergy have been reported. No large series of case studies have been published. OBJECTIVES: We sought to describe demographic, clinical, and immunologic characteristics of patients with mustard hypersensitivity and to determine whether any significant differences exist in age, sex, atopic family history, total immunoglobulin E (IgE) level, and specific IgE to mustard ratio among patients with differing characteristics. METHODS: Twenty-nine patients with a history of mustard allergy underwent skin prick tests with mustard, determination of total IgE, and evaluation of specific IgE to mustard. Skin prick tests with Lolium perenne, Olea europaea, and Artemisia vulgaris were done in patients with symptoms of pollinosis. The aforementioned variables were compared between various subgroups of patients for systemic versus local reactions to mustard, association versus no association with allergies to other vegetable foods, and association versus no association with pollinosis. RESULTS: The male:female ratio was 10:19. Skin prick tests to mustard were positive in all patients. The total IgE geometric mean was 189.3 kU/L. The specific IgE to mustard was positive in all patients (0.7 to > 100 kU/L). Of the 29 patients, 19 (66%) had a systemic reaction after consumption of mustard, and 10 (34%) had a local reaction; 14 (48%) had anaphylaxis. Fifteen patients (52%) had symptoms after ingestion of other vegetable foods, and 15 also had typical symptoms of pollinosis. No significant differences were found in age, sex, atopic family history, total IgE, and specific IgE to mustard between the various subgroups studied. CONCLUSIONS: Most study patients with mustard hypersensitivity had a systemic reaction and had associated pollinosis or allergy to other vegetable foods. Mustard sensitivity should be routinely tested in patients with idiopathic anaphylaxis.