Fiche publication
Date publication
octobre 2018
Journal
Annales de dermatologie et de venereologie
Auteurs
Membres identifiés du Cancéropôle Est :
Pr AUBIN François
,
Pr SEILLES Estelle
,
Dr DAGUINDAU Etienne
,
Dr NARDIN Charlée
Tous les auteurs :
Jacquin-Porretaz C, Castelain F, Daguindau E, Seilles E, Nardin C, Aubin F, Pelletier F
Lien Pubmed
Résumé
Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Laboratory tests are essential in establishing the diagnosis with low levels or abnormal structure and function of C1-INH. Most patients present C1-INH autoantibodies. Furthermore, C1q is reduced in AAE, contrary to HAE. The long-term prognosis is determined by associated hematologic malignancies.
Mots clés
Acquired bradykinin-mediated angioedema, Angiœdème bradykinique acquis, Anticorps anti-C1 inhibiteur, C1-inhibitor antibodies, C1-inhibitor deficiency, Déficit en C1 inhibiteur, Hémopathie maligne, Malignant hemopathy
Référence
Ann Dermatol Venereol. 2018 Oct;145(10):598-602
