Acute angioedema attacks are conventionally treated with antihistamines and steroids, in line with a presumed mechanism of disease involving overwhelming mast-cell degranulation. This approach overlooks a small but important minority of cases in which attacks are bradykinin driven and exhibit poor responsiveness to steroid or anti-histamine therapy. These patients may have a family history of a...

A 34-year-old woman presented to the out-patient clinic with angioedema and type II hereditary angioedema was confirmed immunologically. She also volunteered she had never had a menstrual period and physical examination identified several features of Turner's syndrome. A mosaic karyotype with XY and XO was found on chromosomal analysis and gonadectomy was performed in view of the high risk of g...

Abstract An 81‐year‐old Japanese man presented with a history of recurrent eyelid swelling and purpura on the face, neck, limbs. Because initial clinical presentation was angioedema alone, patient treated an H1‐receptor antagonist tranexamic acid as for idiopathic angioedema. The also experienced dyspnea simultaneously edema face limbs thus taken to emergency room, where laryngeal confirmed fib...

A 44-year-old female is described who developed persistent upper extremity and orolingual dystonia several weeks after suspected onset of central pontine myelinolysis (CPM), later confirmed by characteristic pontine lesions on MRI. No foci of the extrapontine myelinolysis were evident. This case confirms that dystonia may be a late and persistent sequela of CPM and may occur in the absence of v...

Acquired angioedema (AAE) is characterized by acquired deficiency of C1 inhibitor (C1-INH), hyperactivation of the classical pathway of human complement and angioedema symptoms mediated by bradykinin released by inappropriate activation of the contact-kinin system. Angioedema recurs at unpredictable intervals, lasts from two to five days and presents with edema of the skin (face, limbs, genital...

OBJECTIVE To evaluate whether activated partial thromboplastin time (APTT) could be used in the laboratory diagnosis of hereditary or acquired angioedema (HAE or AAE) with and without C1 inhibitor (C1-INH) deficiency. METHODS In a prospective investigation, APTT and other coagulation parameters were determined in 149 adult patients with various types of angioedema and in 26 healthy participan...

BACKGROUND Hereditary angioedema caused by mutations in the factor XII gene is a recently described disease entity that occurs mainly in women. It differs from hereditary angioedema caused by C1 inhibitor deficiency. OBJECTIVE To assess the clinical symptoms, factors triggering acute attacks, and treatments of this disease. METHODS Thirty-five female patients with hereditary angioedema and ...

Introduction The prevalence of urticaria is 15-20%. Women are twice as likely to be affected. Aim To present the epidemiology of urticaria and angioedema in Poland. Material and methods A questionnaire-based survey was conducted on a representative group of 4,897 individuals, aged 15-74 years. Results A total of 11.2% of respondents reported at least one episode of urticaria symptoms in t...

Thromb Haemost 2006; 95: 898–9 Deficiency of the C1-inhibitor (C1-INH) is a very rare cause of angioedema. A hereditary (1) and an acquired form (2, 3) of C1-INH deficiency have been described. Two types of acquired angioedema (AAE) exist: type I is associated with lymphoproliferative disorders, whereas type II is characterized by autoantibodies against C1-INH (4). Clinically, AAE is indistingu...