Hereditary angioedema: A genetic form of angioedema. (Angioedema is also referred to as Quinke's disease.) Persons with it are born lacking an inhibitor protein (called C1 esterase inhibitor) that normally prevents activation of a cascade of ...
Hereditary angioedema
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What is hereditary angioedema?
Hereditary angioedema is a disorder characterized by recurrent episodes of severe swelling (angioedema). The most common areas of the body to develop swelling are the limbs, face, intestinal tract, and airway.
Hereditary angioedema (HAE) is caused by low levels or improper function of a protein called C1 inhibitor. This problem affects the blood vessels.
Weiler CR, van Dellen RG. Genetic test indications and interpretations in patients with hereditary angioedema. Mayo Clin Proc. 2006 Jul;81(7):958-72. Review.
Hereditary angioedema (HAE) on the other hand is a rare, inherited and more serious form of angioedema that is not associated with allergies or hives.
Hereditary angioedema
Hereditary angioedema is the rarest type of angioedema, estimated to affect 1 in 25,000 people.
Hereditary angioedema
Hereditary angioedema is a rare but serious problem with the immune system that is passed down through families. It causes swelling, particularly of the face and airways, and abdominal cramping.
Hereditary angioedema
Transmitted as an autosomal dominant trait, hereditary angioedema is secondary to the absence of an inhibitor of the first component of complement, C1 esterase inhibitor. Severe generalized urticaria can result.
Hereditary angioedema is a rare inherited disorder characterized by recurrent episodes of the accumulation of fluids outside of the blood vessels, blocking the normal flow of blood or lymphatic fluid and causing rapid swelling of tissues in the hands, ...
Hereditary angioedema is characterized by recurrent but self-limited attacks of swelling involving the skin, subcutaneous tissue, upper respiratory tract, or gastrointestinal (GI) tract.
Hereditary angioedema (HAE), a rare condition that is genetic
Acquired angioedema (AAE) ...
Hereditary angioedema is an inherited form of angioedema and is related to low levels or abnormal functioning of certain blood proteins (C1 inhibitors). These inhibitors play a role in regulating how your immune system functions.
Risk factors ...
Hereditary angioedema
-C1 esterase inhibitor concentrate; adults, danazol
Avoid exposure to causative agents
Desensitization to insect venoms
Treat underlying disorders ...
However, hereditary angioedema usually occur earlier in the 20s, while acquired angioedema commonly occurs after the 40s.
See also: Hereditary angioedema
Alternative Names
Angioneurotic edema; Welts
Causes ...
Hepatoma, malignant
Hereditary angioedema
Hereditary dystopic lipidosis
Hereditary motor and sensory neuropathies
Hereditary spherocytosis
Hernia, abdominal
Hernia, groin - child
Hernia, inguinal - child
Hernia-diaphragmatic ...
Hereditary angioedema
Hereditary coproporphyria
Hereditary deafness and nephropathy
Hereditary elliptocytosis
Hereditary fructose intolerance
Hereditary hemorrhagic telangiectasia
Hereditary motor and sensory neuropathy
Hereditary nephritis ...
Hereditary Angioedema see C1 Esterase Inhibitor Deficiency
Hereditary Coproporphyria see Porphyria
Hereditary Haemorrhagic Telangiectasia
Hereditary Hemorrhagic Telangiectasia see Hereditary Haemorrhagic Telangiectasia ...
Clinical manifestations and pathogenesis of hereditary angioedema
Clinical manifestations of food allergy: An overview
Diagnosis of Hymenoptera venom allergy
Diagnostic tools for food allergy
Drug eruptions
Fatal anaphylaxis ...
Hereditary angioedema o This type of angioedema is characterized by recurrent self-limited attacks involving the skin, subcutaneous tissue, upper respiratory tract, or GI tract. Attacks may last from several hours to 2-3 days.
Physical injury can cause angioedema in people with hereditary angioedema (C1 inhibitory deficiency). In these cases, the swelling can last from a few hours to a few days.
C1-inhibitor (Angioedema/Hereditary angioedema) Â- Complement 2 deficiency/Complement 4 deficiency Â- MBL deficiency Â- Properdin deficiency Â- Complement 3 deficiency Â- Terminal complement pathway deficiency Â- Paroxysmal nocturnal hemoglobinuria ...
» Shire Reports Positive Efficacy and Safety Results From FAST-3 Study of FIRAZYR (icatibant) for Acute Attacks of Hereditary Angioedema
» Roche sets up R&D Institute in France to foster collaborative translational research ...
This article does not refer to an uncommon and more serious condition, hereditary angioedema. Hereditary angioedema involves swelling over the entire body, including some internal organs.
Hives and Angioedema Causes ...
(Angioneurotic Edema; Hereditary Angioedema)
by Krisha McCoy, MS
En Español (Spanish Version) ...
Inherited forms (genetic): hereditary angioedema or HEA
Complement mediated (an immune pathway)
Medications (nonallergic, but direct cause, such as ACE inhibitors)
Aspirin
Other anti-inflammatories
Dyes
Chemicals
Emotional stress
Unknown ...
A form of angioedema runs in families and has different triggers, complications, and treatments. This is called hereditary angioedema, and it is not discussed in this article.
Symptoms ...
Angioedema without urticaria occurs with C1 inhibitor deficiency, which can occur as an autosomal dominant characteristic (hereditary angioedema) or be acquired with lymphoproliferative disorders.
This affects the lax tissues around the eyelids, lips, neck and groin. Occasionally, an inherited deficiency of the C1 esterase inhibitor enzyme leads to marked swelling without urticaria. This rare condition is called hereditary angioedema.
glomerulonephritis), due to defective clearance of antigen-antibody complexes (see Table 3: Immunodeficiency Disorders: Primary Immunodeficiency Disorders ), are the most serious consequences. One of these deficiencies causes hereditary angioedema.
It is confirmed by finding abnormally low levels of C1 esterase inhibitor in the blood. Treatment options include antihistamines and male steroids (androgens) that can also prevent the recurrent attacks. Also called hereditary angioedema.
inherited conditions, such as a disorder known as hereditary angioedema. This condition can cause occasional bouts of severe facial swelling.
a tumor or cancer in or under the skin of the face, such as skin cancer.
 See also: Angioedema, Symptom, Allergy, Hives, Immune System
  
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