The spectrum and treatment of angioedema.

Angioedema manifests as episodes of localized swelling in the dermis and submucosa. The key to successful management is detection and avoidance of triggers, early recognition of attacks, and aggressive airway management when warranted. Review of a patient's medication list may identify drugs that include angiotensin-converting enzyme inhibitor or angiotensin receptor blockers as the cause. Initial treatment in a patient presenting with most forms of angioedema includes antihistamines and glucocorticoids if required. Epinephrine should be administered if there is concern for laryngeal edema. Patients who have a known history of hereditary angioedema should receive C1 esterase inhibitor concentrate or fresh-frozen plasma.

Antigen challenge induces a supraglottic but not a subglottic edema in the rat larynx.

OBJECTIVE: To examine the reaction of mast cells in the larynx of sensitized rats after allergen challenge and determine whether a type I hypersensitivity reaction can elicit a supraglottic and subglottic edema. STUDY DESIGN: Rats were sensitized with ovalbumin (OA) and challenged 3 weeks later by intravenous injection of either OA or NaCl and perfused with glutaraldehyde. The larynges were removed and processed for light and electron microscopy. Sections from the epiglottis and subglottic region were examined. RESULTS: In challenged animals, the connective tissue mast cells (CTMC) in the epiglottic submucosa, but not the mucosal mast cells (MMC) in epithelium, were significantly reduced compared with the case of controls, and the connective tissue was edematous. In the subglottic region, the number of both MMC and CTMC was reduced (albeit not significantly), and there was no edema in the lamina propria. CONCLUSIONS: Allergen challenge induces a marked edema in the epiglottis but not in the subglottic area. SIGNIFICANCE: The results indicate that the supraglottic and subglottic regions react differently to allergen challenge.

[Hereditary or acquired angioedema caused by functional deficiency of C1 inhibitor--a still unfamiliar disease picture]. / Hereditäres oder erworbenes Angioödem durch funktionellen Mangel von C1-Inhibitor--ein immer noch zu wenig bekanntes Krankheitsbild.

Hereditary angioneurotic oedema or hereditary angiooedema (HAE) and acquired angiooedema (AAE) are disorders of the C1-inhibitor (C1-INH) protein, caused by the lack, dysfunction or exhaustion of the C1-INH molecule. Inadequate function of C1-INH results in inappropriate control of various enzymes of the fibrinolytic, complement and kinin systems as well as of factor XII, being the initial enzyme of the kinin and contact coagulation systems. As C1-INH functional deficiency is rare and the clinical manifestation little known, even nowadays the most feared complication of the deficiency may evolve: death from acute airway obstruction. Patients deficient in C1-INH function whose clinical manifestations are misinterpreted as allergic angiooedema are most at risk for fatal laryngeal oedema. The therapy of allergic and C1-INH-related angiooedema is fundamentally different. The background for the hereditary form of inadequate C1-INH function is a gene defect. The predominant primary underlying disease of the acquired form of the deficiency (AAE) is a lymphoproliferative process.

The role of allergy in the etiopathogenesis of laryngeal mucosal lesions.

The results of this study showed that allergy is an important factor in the etiopathogenesis of laryngeal mucosal lesions. Despite adequate treatment, no other unfavourable factors appeared to have significant influence on the results of the treatment. It seems that hypersensitivity to different inhalatory and nutritional allergens make laryngeal mucosa more susceptible for adverse action of other factors: vocal misuse, gastroesophageal reflux (GER), smoking, irritants in the surrounding microclimate, endocrinologic disorders, etc. Acting together, all these factors cause the development of laryngeal mucosal lesions. In the treatment of noninfectious laryngitis, vocal cord nodules, polyps or Reinke's edema, all the stated adverse factors should be identified and suitably diminished or eliminated. Allergy (Ig-E-mediated and non-IgE-mediated) should be considered as only one of the etiopathogenetic factors.

The diagnosis of hymenoptera hypersensitivity.

Venom skin testing was performed in 216 patients with histories of systemic or local reactions following insect stings. Seventy-six percent of the patients with histories of systemic reactions and 77% of the patients with histories of local reactions had positive skin tests up to 1 mcg/ml. The majority of vespid sensitive patients had positive skin tests to all the vespid species. Significant (p less than .001) cross-reactivity is suggested by regression analysis. This study confirms the value and safety of venom skin testing in hymenoptera hypersensitivity. The implications for venom immunotherapy of the findings are also discussed.

Angioneurotic edema. A review of modern concepts.

We review the subject of angioneurotic edema with special emphasis on the more clearly defined entity of hereditary angioneurotic edema. The clinical presentations of the various forms of angioneurotic edema are discussed. Attention is directed toward the attempts that have been made to define this group of diseases in terms of their underlying biochemical mechanisms. A simplified schema of the serum complement system is presented. We conclude that only after we understand such biochemical interactions will we able to effect a more definitive form of therapy for the angioneurotic edemas.