title: Idiopathic histaminergic angioedema without wheals: a case series of 31 patients.
abstract: Idiopathic histaminergic acquired angioedema (IH-AAE) is a common cause of recurrent angioedema without wheals. It is a mast cell-mediated disease thought to belong to the same clinical entity as chronic urticaria (CU). The objective of this study was to describe the clinical and epidemiological characteristics of IH-AAE patients. From 2014 to 2015, 534 patients were seen at our national reference centre for angioedema and/or urticaria. Among them, we identified 31 patients with idiopathic histaminergic acquired angioedema without wheals (IH-AAE). Thirty-one patients (15 men and 16 women) with a mean age of 50 years met the criteria for IH-AAE. The average delay in diagnosis was 6·3 years. A history of allergy was found in 12 patients (38·7%), nine suffering from allergic rhinitis. The mean duration of attacks was 28·1 h. The AE attack was located in the upper respiratory tract in 54·8% of cases (17 patients). A lingual location was found in 29% of patients. Men were more likely than women to have an upper airway involvement. No intubations or admissions to intensive care units were reported. The dosage of anti-histamines to control the symptoms was onefold the recommended dose in 51·6% of patients (16 patients), twofold in 32% (10 patients) and three-fourfold in 16·1% (five patients). IH-AAE is characterized by an important delay in diagnosis, a frequent involvement of the upper airway and a benign course during attacks. As in CU, a trial of up to fourfold dose of H1-anti-histamines may be necessary to control symptoms.
authors.affiliation: Department of Internal Medicine, Grenoble University Hospital.|Grenoble Alps University.
authors.forename: C
authors.initials: C
authors.lastname: Faisant
authors.affiliation: Department of Internal Medicine, Grenoble University Hospital.|French National Reference Centre for Angioedema (CREAK).
authors.forename: I
authors.initials: I
authors.lastname: Boccon-Gibod
authors.affiliation: Department of Internal Medicine, Grenoble University Hospital.|Grenoble Alps University.
authors.forename: C
authors.initials: C
authors.lastname: Mansard
authors.affiliation: Immunology Laboratory.
authors.forename: C
authors.initials: C
authors.lastname: Dumestre Perard
authors.affiliation: Department of Dermatology and Allergology, Grenoble University Hospital.
authors.forename: P
authors.initials: P
authors.lastname: Pralong
authors.affiliation: Department of Dermatology and Allergology, Grenoble University Hospital.
authors.forename: C
authors.initials: C
authors.lastname: Chatain
authors.affiliation: Department of Internal Medicine, Grenoble University Hospital.|Grenoble Alps University.|French National Reference Centre for Angioedema (CREAK).
authors.forename: A
authors.initials: A
authors.lastname: Deroux
authors.affiliation: Department of Internal Medicine, Grenoble University Hospital.|Grenoble Alps University.|French National Reference Centre for Angioedema (CREAK).|Inserm-UJF-CEA-CNRS unit 1036, IRTSV, CEA Grenoble, Grenoble, France.
authors.forename: L
authors.initials: L
authors.lastname: Bouillet
mesh_terms: D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology* / Q000473:pathology; D057210:Delayed Diagnosis; D054796:Drug Dosage Calculations; D005260:Female; D006632:Histamine / Q000276:immunology / Q000378:metabolism; D006633:Histamine Antagonists / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012137:Respiratory System / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D012189:Retrospective Studies; D014581:Urticaria / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology* / Q000473:pathology
chemical_list: D006633:Histamine Antagonists; D006632:Histamine
keywords: Allergy; Inflammation; Mast Cells; Skin