There are several types of angioedema, each with different causes. The most common is an allergic reaction to something in the environment. Certain medications, infections, and diseases also can cause angioedema.
Some people have a condition called hereditary angioedema (HAE), which has a genetic component.
This article looks at the various types of angioedema and their causes. It explains what happens in the body when swelling occurs and describes the wide variety of triggers for this reaction.
Angioedemais not the same thing asurticaria, or hives, which is swelling of the surface of the skin rather than the tissue beneath it. Angioedema can occur along with hives.
Verywell
Common Causes
There are two types of angioedema—one occurs when the immune system releases histamine, the other when the body releases peptides calledbradykininsthat regulate many important body functions.
Histaminergic
Most of the time, angioedema occurs when the immune system mistakes an otherwise harmless substance as a danger to the body. In defense, it releases a chemical called histamine into the bloodstream.
Allergens can be triggers of such reactions. Among the most common allergens associated with angioedema are:
There are a handful of less obvious causes of histaminergic angioedema. Some people experience swelling when exposed to extreme heat or cold, sunlight,or vibration—for example, while using a lawnmower, riding in a bumpy vehicle, or even toweling off after a shower.
Non-Histaminergic
These substances play many roles in the body. One is to dilate (widen) blood vessels. This is important for several body functions, such as keepingblood pressure at normal levels.
If bradykinins are released when they aren’t needed, their effect on the blood vessels can cause them to leak into surrounding tissue and cause swelling. Most often, the feet, hands, genitals, face, lips, larynx, and gastrointestinal (GI) tract are affected.
ACE inhibitors, which treat high blood pressure, heart attack, failure, and nephropathy, are common causes of drug-induced non-histaminergic angioedema.
Sometimes the cause of angioedema isn’t clear, meaning it’s “idiopathic.”In most cases, the face, mouth, and tongue are affected.
Recap
Genetics
Some people who experience bradykinin-mediated angioedemahave a gene mutation that makes them susceptible to triggers. This is called hereditary angioedema and an autosomal dominant disorder, meaning you must inherit the responsible gene from only one parent.
Genetics also may factor in vibratory angioedema. Autosomal dominant vibratory urticaria has been linked to a mutation in the ADGRE2 gene, which allows mast cells to release histamine more easily.
HAE is rare, occurring in one of every 50,000 people, and is often only suspected if someone with angioedema does not respond to antihistamines or has a family history of angioedema.
Summary
Angioedema is the swelling of tissue underneath the skin. It occurs when either histamine, an immune system chemical, or peptides called bradykinins are mistakenly released into the bloodstream, where they cause fluid to leak from blood vessels.
There is a wide variety of reasons this can happen, ranging from exposure to an allergen that triggers histamine to certain medications or medical conditions or procedures that trigger bradykinins. A tendency to develop angioedema also can be inherited.
A Word From Verywell
If you or someone else experiences sudden swelling or any symptoms of angioedema, you should get medical attention. Angioedema that affects the GI tract can cause violent vomiting, intense pain, and dehydration.
When swelling affects the face, mouth, tongue, larynx, or throat, it can restrict air flow to the lungs and is a medical emergency.
Frequently Asked Questions
Learn MorePossible Causes of Swollen Lips
Chronic and relapsing angioedema is often linked toautoimmune diseases, like lupus andHashimoto’s thyroiditis. Some studies suggest that as many as 30% to 50% of idiopathic angioedema (meaning angioedema of unknown origin) may be associated with some autoimmune diseases. In such cases, itchyhives (urticaria)commonly accompany the deeper tissue swelling.
Pressure angioedema develops when steady pressure applied to an area of the skin causes immediate or delayed deep tissue swelling. It is believed to be autoimmune in nature. Examples include wearing tight clothes or sitting for long periods, during which sustained pressure causes mast cells found in vascular tissues to break open and release histamine. Extreme vibrations can do the same.
Angioedema can occur due to exposure to certain drugs:
How Angioedema Is Diagnosed
Regardless of what causes it, angioedema in the GI tract can cause violent vomiting, intense pain, and dehydration. When swelling affects the face, mouth, tongue, larynx, or throat, it can restrict the flow of air to the lungs and is a medical emergency.
10 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Bova M, De Feo G, Parente R, et al.Hereditary and acquired angioedema: heterogeneity of pathogenesis and clinical phenotypes.Int Arch Allergy Immunol. 2018;175(3):126-135. doi:10.1159/000486312Bernstein JA, Moellman J.Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema.Int J Emerg Med. 2012;5(1):39. doi:10.1186/1865-1380-5-39Tarbox JA, Bansal A, Peiris AN.Angioedema.JAMA. 2018;319(19):2054. doi:10.1001/jama.2018.4860National Library of Medicine: MedlinePlus.Angioedema.National Library of Medicine: MedlinePlus.Vibratory urticaria.Obtułowicz K.Bradykinin-mediated angioedema.Pol Arch Med Wewn. 2016;126(1-2):76-85. doi:10.20452/pamw.3273National Library of Medicine: MedlinePlus.Hereditary angioedema.Caccia S, Suffritti C, Cicardi M.Pathophysiology of hereditary angioedema.Pediat Allergy Immunol Pulmonol. 2014;27(4):159-63. doi:10.1089/ped.2014.0425Cho CB, Stutes SA, Altrich ML, Ardoin SP, Phillips G, Ogbogu PU.Autoantibodies in chronic idiopathic urticaria and nonurticarial systemic autoimmune disorders.Ann Allergy Asthma Immunol. 2013;110(1):29-33. doi:10.1016/j.anai.2012.10.020Bernstein JA, Cremonesi P, Hoffmann TK, Hollingsworth J.Angioedema in the emergency department: a practical guide to differential diagnosis and management.Int J Emerg Med. 2017;10(1):15. doi:10.1186/s12245-017-0141-z
10 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Bova M, De Feo G, Parente R, et al.Hereditary and acquired angioedema: heterogeneity of pathogenesis and clinical phenotypes.Int Arch Allergy Immunol. 2018;175(3):126-135. doi:10.1159/000486312Bernstein JA, Moellman J.Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema.Int J Emerg Med. 2012;5(1):39. doi:10.1186/1865-1380-5-39Tarbox JA, Bansal A, Peiris AN.Angioedema.JAMA. 2018;319(19):2054. doi:10.1001/jama.2018.4860National Library of Medicine: MedlinePlus.Angioedema.National Library of Medicine: MedlinePlus.Vibratory urticaria.Obtułowicz K.Bradykinin-mediated angioedema.Pol Arch Med Wewn. 2016;126(1-2):76-85. doi:10.20452/pamw.3273National Library of Medicine: MedlinePlus.Hereditary angioedema.Caccia S, Suffritti C, Cicardi M.Pathophysiology of hereditary angioedema.Pediat Allergy Immunol Pulmonol. 2014;27(4):159-63. doi:10.1089/ped.2014.0425Cho CB, Stutes SA, Altrich ML, Ardoin SP, Phillips G, Ogbogu PU.Autoantibodies in chronic idiopathic urticaria and nonurticarial systemic autoimmune disorders.Ann Allergy Asthma Immunol. 2013;110(1):29-33. doi:10.1016/j.anai.2012.10.020Bernstein JA, Cremonesi P, Hoffmann TK, Hollingsworth J.Angioedema in the emergency department: a practical guide to differential diagnosis and management.Int J Emerg Med. 2017;10(1):15. doi:10.1186/s12245-017-0141-z
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Bova M, De Feo G, Parente R, et al.Hereditary and acquired angioedema: heterogeneity of pathogenesis and clinical phenotypes.Int Arch Allergy Immunol. 2018;175(3):126-135. doi:10.1159/000486312Bernstein JA, Moellman J.Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema.Int J Emerg Med. 2012;5(1):39. doi:10.1186/1865-1380-5-39Tarbox JA, Bansal A, Peiris AN.Angioedema.JAMA. 2018;319(19):2054. doi:10.1001/jama.2018.4860National Library of Medicine: MedlinePlus.Angioedema.National Library of Medicine: MedlinePlus.Vibratory urticaria.Obtułowicz K.Bradykinin-mediated angioedema.Pol Arch Med Wewn. 2016;126(1-2):76-85. doi:10.20452/pamw.3273National Library of Medicine: MedlinePlus.Hereditary angioedema.Caccia S, Suffritti C, Cicardi M.Pathophysiology of hereditary angioedema.Pediat Allergy Immunol Pulmonol. 2014;27(4):159-63. doi:10.1089/ped.2014.0425Cho CB, Stutes SA, Altrich ML, Ardoin SP, Phillips G, Ogbogu PU.Autoantibodies in chronic idiopathic urticaria and nonurticarial systemic autoimmune disorders.Ann Allergy Asthma Immunol. 2013;110(1):29-33. doi:10.1016/j.anai.2012.10.020Bernstein JA, Cremonesi P, Hoffmann TK, Hollingsworth J.Angioedema in the emergency department: a practical guide to differential diagnosis and management.Int J Emerg Med. 2017;10(1):15. doi:10.1186/s12245-017-0141-z
Bova M, De Feo G, Parente R, et al.Hereditary and acquired angioedema: heterogeneity of pathogenesis and clinical phenotypes.Int Arch Allergy Immunol. 2018;175(3):126-135. doi:10.1159/000486312
Bernstein JA, Moellman J.Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema.Int J Emerg Med. 2012;5(1):39. doi:10.1186/1865-1380-5-39
Tarbox JA, Bansal A, Peiris AN.Angioedema.JAMA. 2018;319(19):2054. doi:10.1001/jama.2018.4860
National Library of Medicine: MedlinePlus.Angioedema.
National Library of Medicine: MedlinePlus.Vibratory urticaria.
Obtułowicz K.Bradykinin-mediated angioedema.Pol Arch Med Wewn. 2016;126(1-2):76-85. doi:10.20452/pamw.3273
National Library of Medicine: MedlinePlus.Hereditary angioedema.
Caccia S, Suffritti C, Cicardi M.Pathophysiology of hereditary angioedema.Pediat Allergy Immunol Pulmonol. 2014;27(4):159-63. doi:10.1089/ped.2014.0425
Cho CB, Stutes SA, Altrich ML, Ardoin SP, Phillips G, Ogbogu PU.Autoantibodies in chronic idiopathic urticaria and nonurticarial systemic autoimmune disorders.Ann Allergy Asthma Immunol. 2013;110(1):29-33. doi:10.1016/j.anai.2012.10.020
Bernstein JA, Cremonesi P, Hoffmann TK, Hollingsworth J.Angioedema in the emergency department: a practical guide to differential diagnosis and management.Int J Emerg Med. 2017;10(1):15. doi:10.1186/s12245-017-0141-z
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