Hereditary angioedema (HAE) is a chronic condition that may cause painful swelling in your skin, abdomen, and other parts of your body.

Hereditary angioedema (HAE) causes episodes of angioedema, a deep swelling that occurs under your skin or mucosal membranes. This swelling may affect various parts of your body, such as the:

  • face, hands, feet, limbs, or genitals
  • gastrointestinal tract
  • respiratory tract

HAE symptoms may range from uncomfortable to extremely painful and debilitating.

Certain triggers may cause episodes of symptoms, although episodes may also occur with no known trigger. Common triggers include injuries, infections, and medical or dental procedures.

The average number of episodes in which people have active HAE symptoms ranges from 1 to 26 per year, although some have as many as 100 episodes per year.

Treatment may help reduce symptom frequency and ease pain and other symptoms when they occur.

Read on to learn more about HAE pain and how to manage it.

HAE may cause swelling of tissues under your skin, which may affect various parts of your body.

Common locations include the:

  • face
  • hands
  • feet
  • limbs

Swelling under the skin may also occur on other parts of the body, such as the back, chest, or abdomen.

Skin swelling may be painful, especially if it’s severe. It may limit your ability to move the affected body part and perform certain activities. For example, hand swelling can make it challenging to type or write.

Abdominal pain affects up to 93% of people with HAE, while this type of pain is recurring for roughly 80% of those with this condition.

Abdominal pain from HAE occurs when the mucosal tissue that lines your gastrointestinal tract swells.

It may occur on its own or with other symptoms, such as:

  • cramping
  • nausea
  • vomiting
  • diarrhea
  • abdominal distension, or swelling
  • swelling in other parts of your body

Sometimes doctors misdiagnose the cause of HAE abdominal pain as appendicitis or another gastrointestinal condition. This may lead to unnecessary surgery or delay HAE diagnosis and treatment.

HAE may cause painful swelling in the upper airway, including the:

  • larynx
  • throat
  • tongue

Other potential symptoms of airway swelling include:

  • hoarseness
  • difficulty swallowing
  • difficulty breathing
  • choking sensation

Swelling in your upper airway is an emergency that can cause life threatening breathing difficulties.

HAE symptoms may affect tissues on or around the:

  • penis
  • scrotum
  • vulva
  • vagina
  • perineum
  • buttocks

A 2024 study surveyed 42 adults with HAE symptoms and found that roughly 80% had experienced genital symptoms. Over 40% of survey participants said that sexual activity had triggered an episode. Ten of those participants reported genital pain, tenderness, or swelling during the symptom episode.

Let your doctor know if you have a family history of HAE or think you may have symptoms of it. They will ask questions about your medical history and order tests to diagnose HAE.

Follow your treatment plan to manage HAE triggers, limit the frequency of symptoms, and treat symptoms when they develop. Let your doctor know if you have questions or concerns about your treatment.

If you develop swelling in your upper airway, take your prescribed emergency medication for symptoms, and contact emergency medical services (EMS) or go to the emergency department. Swelling in your upper airway can cause life threatening breathing difficulties that may require hospitalization to monitor and treat.

Tissue swelling from HAE may cause pain and other symptoms that may lower your quality of life and limit your ability to perform certain activities. Swelling in your upper airway can cause life threatening breathing difficulties.

Getting treatment may help improve your outlook with HAE.

Your doctor may prescribe multiple medications, including:

  • preventive or prophylactic medication to reduce the frequency of symptoms
  • on-demand or acute medication to treat symptoms when they occur

Your doctor can also help you avoid or manage triggers that may cause symptom episodes.

Hereditary angioedema (HAE) may cause painful swelling in the face, limbs, abdomen, and other parts of your body.

Your doctor may prescribe medication to reduce and treat symptom episodes. Take on-demand medication as soon as possible when your symptoms develop.

Contact EMS or go to the emergency department if you think you may have swelling in your upper airway, including your larynx, throat, or tongue. This is a life threatening emergency that requires immediate treatment.