Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare hereditary angioedema characterized by potentially life-threatening episodes of subcutaneous and/or submucosal edema without urticaria and with normal levels and function of C1 esterase inhibitor. Patients present with prolonged attacks which last for approximately two to five days and may include nonpitting edema of the skin, severe abdominal symptoms such as pain and swelling, and/or respiratory distress due to upper respiratory airways involvement. Affected locations and frequency of attacks differ slightly between subtypes. Estrogen-containing oral contraceptives and pregnancy are precipitating factors, especially in patients with a factor XII mutation. Disease data Classification Disease Synonyms HAE with normal C1 inhibitor HAE z prawidłowym poziomem C1Inh HAE z prawidłowym poziomem inhibitora C1 Dziedziczny obrzęk naczynioruchowy z prawidłowym poziomem inhibitora C1 Dziedziczny obrzęk angioneurotyczny z prawidłowym poziomem C1Inh Dziedziczny obrzęk angioneurotyczny z prawidłowym poziomem inhibitora C1 HAE with normal C1Inh Hereditary angioedema with normal C1 inhibitor Hereditary angioneurotic edema with normal C1 inhibitor Hereditary angioneurotic edema with normal C1Inh ORPHA code 528647 OMIM code - ICD10 code D84.1 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl