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[[Angioedema]] is a medical condition characterized by a sudden, rapid swelling of the deep layers of the skin and subcutaneous tissues. This condition is a result of the transient vascular leakage of serous fluid. Although it can occur anywhere on the body, angioedema commonly affects the face, lips, tongue, throat, and extremities.<ref>{{Cite journal|last=Bernstein|first=Jonathan A.|title=Angioedema in the emergency department: a practical guide to differential diagnosis and management|journal=International Journal of Emergency Medicine|year=2017|volume=10|issue=1|pages=15|doi=10.1186/s12245-017-0141-0|pmid=28405953|pmc=PMC5387545}}</ref>
Angioedema
[[File:AngioedemaFra.JPG|thumb|AngioedemaFra]]
== Pathophysiology ==


Angioedema results from the dilation and increased permeability of blood vessels in deep dermal, subcutaneous, or submucosal tissues. This process can be triggered by various factors, including an allergic reaction, hereditary factors, or as a side effect of certain medications, such as angiotensin-converting enzyme (ACE) inhibitors.<ref>{{Cite journal|last=Cicardi|first=M.|title=Angioedema due to angiotensin-converting enzyme inhibitors|journal=Immunology and Allergy Clinics of North America|year=2006|volume=26|issue=4|pages=739–750|doi=10.1016/j.iac.2006.09.008|pmid=17085285}}</ref>
[[File:Angioedema2010.JPG|thumb|Angioedema of the lips]]
[[File:Angioedema2010.JPG|thumb|Angioedema2010|left]]
[[File:Angioedema2013.JPG|thumb|Angioedema affecting the face]]
== Clinical Presentation ==
[[File:Angioedema of the face.jpg|thumb|Severe angioedema of the face]]


The hallmark of angioedema is the rapid onset of swelling involving the skin, mucosa, and submucosal tissues. Commonly affected areas include the face, lips, tongue, larynx, abdomen, and extremities. In severe cases, it can lead to difficulty breathing or swallowing, which can be life-threatening and requires immediate medical attention.<ref>{{Cite journal|last=Banerji|first=Aleena|title=Current treatment of hereditary angioedema: An update on clinical studies|journal=Allergy and Asthma Proceedings|year=2010|volume=31|issue=4|pages=286–290|doi=10.2500/aap.2010.31.3336|pmid=20615327}}</ref>
'''Angioedema''' is a medical condition characterized by the rapid swelling of the dermis, subcutaneous tissue, mucosa, and submucosal tissues. It is similar to [[urticaria]] (hives), but angioedema occurs deeper in the skin. The swelling is often localized and can affect various parts of the body, including the face, lips, tongue, throat, and extremities.
[[File:Swollen hand during a hereditary angioedema attack..jpg|thumb|Swollen hand during a hereditary angioedema attack.]]
== Diagnosis and Management ==


Diagnosis of angioedema involves a thorough medical history and physical examination, alongside laboratory investigations, if needed, to rule out underlying causes. Management depends on the severity and the cause of the condition. Treatment may include antihistamines, corticosteroids, epinephrine for acute episodes, and certain types of blood pressure medications like ACE inhibitors should be avoided. In cases of hereditary angioedema, therapies to regulate the protein C1-INH might be used.<ref>{{Cite journal|last=Zuraw|first=Bruce L.|title=Hereditary angioedema with normal C1 inhibitor: Four types and counting|journal=The Journal of Allergy and Clinical Immunology|year=2018|volume=141|issue=3|pages=884–885|doi=10.1016/j.jaci.2017.11.034|pmid=29241729}}</ref>
==Etiology==
Angioedema can be classified into several types based on its etiology:


== References ==
* '''Allergic Angioedema''': This type is often associated with allergic reactions to foods, medications, insect stings, or other allergens. It is mediated by [[immunoglobulin E]] (IgE) and is often accompanied by urticaria.
 
* '''Hereditary Angioedema (HAE)''': A rare genetic disorder caused by a deficiency or dysfunction of the C1 inhibitor protein, leading to excessive production of bradykinin, a peptide that increases vascular permeability.
 
* '''Acquired Angioedema''': Similar to hereditary angioedema but occurs later in life and is often associated with autoimmune diseases or lymphoproliferative disorders.
 
* '''Drug-Induced Angioedema''': Commonly associated with the use of [[angiotensin-converting enzyme inhibitors]] (ACE inhibitors), which can increase bradykinin levels.
 
* '''Idiopathic Angioedema''': When no specific cause can be identified.
 
==Pathophysiology==
The pathophysiology of angioedema involves the release of mediators that increase vascular permeability, leading to fluid leakage into the interstitial tissues. In allergic angioedema, histamine is the primary mediator, while in bradykinin-mediated angioedema (such as HAE), bradykinin is the key mediator.
 
==Clinical Presentation==
Patients with angioedema typically present with sudden onset of swelling in the affected areas. The swelling is usually non-pitting and can be painful or itchy. In severe cases, especially when the airway is involved, angioedema can be life-threatening and requires immediate medical attention.
 
==Diagnosis==
Diagnosis of angioedema is primarily clinical, based on the characteristic swelling and patient history. Laboratory tests may include:
 
* Measurement of C4 and C1 inhibitor levels for hereditary angioedema.
* Allergy testing for allergic angioedema.
 
==Management==
The management of angioedema depends on the underlying cause:
 
* '''Acute Management''': Involves airway protection, administration of antihistamines, corticosteroids, and epinephrine for allergic angioedema.
 
* '''Long-term Management''': For hereditary angioedema, prophylactic treatments such as C1 inhibitor concentrates, bradykinin receptor antagonists, or kallikrein inhibitors may be used.
 
==Prognosis==
The prognosis of angioedema varies depending on the type and severity. Allergic angioedema often resolves with treatment, while hereditary angioedema requires ongoing management to prevent attacks.
 
==Also see==
* [[Urticaria]]
* [[Anaphylaxis]]
* [[Bradykinin]]
* [[C1 inhibitor]]
 
{{Medical conditions}}


{{Reflist}}
{{stub}}
[[Category:Skin conditions]]
[[Category:Emergency medicine]]
[[Category:Immunology]]
[[Category:Allergology]]
[[Category:Allergology]]
[[Category:Dermatology]]
[[Category:Genetic disorders]]

Revision as of 02:58, 11 December 2024

Angioedema

Angioedema of the lips
Angioedema affecting the face
Severe angioedema of the face

Angioedema is a medical condition characterized by the rapid swelling of the dermis, subcutaneous tissue, mucosa, and submucosal tissues. It is similar to urticaria (hives), but angioedema occurs deeper in the skin. The swelling is often localized and can affect various parts of the body, including the face, lips, tongue, throat, and extremities.

Etiology

Angioedema can be classified into several types based on its etiology:

  • Allergic Angioedema: This type is often associated with allergic reactions to foods, medications, insect stings, or other allergens. It is mediated by immunoglobulin E (IgE) and is often accompanied by urticaria.
  • Hereditary Angioedema (HAE): A rare genetic disorder caused by a deficiency or dysfunction of the C1 inhibitor protein, leading to excessive production of bradykinin, a peptide that increases vascular permeability.
  • Acquired Angioedema: Similar to hereditary angioedema but occurs later in life and is often associated with autoimmune diseases or lymphoproliferative disorders.
  • Idiopathic Angioedema: When no specific cause can be identified.

Pathophysiology

The pathophysiology of angioedema involves the release of mediators that increase vascular permeability, leading to fluid leakage into the interstitial tissues. In allergic angioedema, histamine is the primary mediator, while in bradykinin-mediated angioedema (such as HAE), bradykinin is the key mediator.

Clinical Presentation

Patients with angioedema typically present with sudden onset of swelling in the affected areas. The swelling is usually non-pitting and can be painful or itchy. In severe cases, especially when the airway is involved, angioedema can be life-threatening and requires immediate medical attention.

Diagnosis

Diagnosis of angioedema is primarily clinical, based on the characteristic swelling and patient history. Laboratory tests may include:

  • Measurement of C4 and C1 inhibitor levels for hereditary angioedema.
  • Allergy testing for allergic angioedema.

Management

The management of angioedema depends on the underlying cause:

  • Acute Management: Involves airway protection, administration of antihistamines, corticosteroids, and epinephrine for allergic angioedema.
  • Long-term Management: For hereditary angioedema, prophylactic treatments such as C1 inhibitor concentrates, bradykinin receptor antagonists, or kallikrein inhibitors may be used.

Prognosis

The prognosis of angioedema varies depending on the type and severity. Allergic angioedema often resolves with treatment, while hereditary angioedema requires ongoing management to prevent attacks.

Also see