Synonyms: HAE with normal C1 inhibitor | HAE with normal C1Inh | Hereditary angioedema with normal C1 inhibitor | Hereditary angioneurotic edema with normal C1 inhibitor | Hereditary angioneurotic edema with normal C1Inh
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.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2026
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Hereditary angioedema with normal C1Inh?
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Advocacy Organizations
Advocacy & Awareness for Immune Disorders Association (AAIDA)
AAIDA exists to provide support and advocate for the greater than 25 million Americans living with immune disorders. AAIDA works to promote research and create better awareness of immune disorders.
Cookies4Cures
Funding research into rare pediatric diseases
Mozambique Hereditary Angioedema Association
The Associação Moçambicana de Angioedema Hereditário (AMAEH) is dedicated to improving the lives of people living with Hereditary Angioedema (HAE) in Mozambique. We work to promote early diagnosis, equitable access to treatment, patient education, and psychosocial support, while advocating for the rights of individuals and families affected by HAE.
US Hereditary Angioedema Association
To lead a nationwide advocacy movement that focuses on increasing HAE awareness and education, empowering access to suitable treatment, and fostering ground-breaking research that includes searching for a cure.
Unfixed
Unfixed produces media that elevates stories of people living with chronic illness, rare disease and disability. The Unfixed portfolio of projects demonstrates that living well is not about eradicating our wounds and weaknesses but understanding how they complete our identities and equip us to help others. Current and past productions include multiple mini-series, a podcast and feature film.
accessia health
Accessia Health, a national charitable patient assistance organization, is dedicated to eliminating healthcare barriers for people with rare or chronic health conditions. Comprehensive services include personalized case management, financial assistance, education, and legal aid support. Our flexible funding model goes beyond copays, allowing individuals to pay for other essential medical expenses
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Clinical Trials
For a list of clinical trials in this disease area, please click here.