HAE: Recurrent Swelling Without Hives
Hereditary angioedema, or HAE, is a rare genetic condition that causes repeated attacks of swelling in the skin, digestive tract and upper airway. It is estimated to affect about 1 in 50,000 people. Unlike allergic angioedema, HAE is not caused by histamine and does not come with hives.
Types of HAE
- Type I: low levels of C1 inhibitor
- Type II: normal levels but poorly working C1 inhibitor
- Type III (normal C1 inhibitor): often linked to other gene changes and sensitive to estrogen
Symptoms
Swelling that is not itchy, often of the hands, feet, face, lips or genitals, lasts two to five days. Abdominal attacks cause severe cramping pain, nausea and vomiting. Swelling of the throat can be life-threatening. Attacks may be triggered by trauma, dental work, infection, stress, or estrogen-containing medicines. Compare with general angioedema symptoms.
Diagnosis
Blood tests measure C4, C1 inhibitor level and function. Because it is inherited, family history matters, although new mutations can occur. Antihistamines, steroids and often epinephrine do not stop HAE attacks.
Treatment
Care is managed by an allergist or immunologist. On-demand treatments include C1 inhibitor concentrate, icatibant and ecallantide. Long-term preventive options include lanadelumab, berotralstat, subcutaneous or intravenous C1 inhibitor, and newer approved agents. See angioedema treatment.
When to Call 911
Call 911 right away if hives or swelling occur with difficulty breathing or swallowing, a tight or closing throat, a swollen tongue, lips or face, wheezing, faintness, or a sudden feeling of impending doom. Use an epinephrine auto-injector first if one has been prescribed to you, then call for emergency help.
When to See a Doctor
See a doctor if your symptoms last more than a few days, keep coming back, cover large areas, or interfere with sleep or daily life. Dr. Tiffany Young’s Hives Treatment Center in San Francisco offers online and in-person consultations for patients with hereditary angioedema.
Related Reading
You may also find these guides helpful: angioedema and angioedema causes.
Frequently Asked Questions About Hereditary Angioedema
Is HAE contagious?
No. It is inherited, not infectious.
Does HAE cause hives?
No. Hives are not part of HAE.
Can HAE be life-threatening?
Yes, throat swelling needs emergency treatment.
Get Specialist Care
HAE needs a specialist diagnosis and an emergency action plan. Visit our hives treatment page to see the latest treatment options for your outbreak.
This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis or treatment. If you think you are having a medical emergency, call 911.
