Sandbox:Sukaina

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sukaina Furniturewala, MBBS[2]

ANGIODEMA

Angiodema is defined as the rapid swelling beneath the deeper layers of the skin such as the dermis and the mucous membranes.

• It encompasses a variety of causes such as allergies( food and drugs), hereditary and acquired causes.

• Angiodema affects upto 20% of the population with an estimate of 10-20 % of the population experiences at least one episode of angiodema in their lifetime.
• Most cases of angiodema are associated are self limiting with low mortality rates. The case fatality seen in angiodema can be attributed to cases of hereditary angiodema, an autosomal dominant condition that can cause significant laryngeal edema, a life threatening complication and hallmark of this condition.

CAUSES

1. Allergen associated angiodema

• most prevalent cause of angiodema that presents suddenly within minutes to seconds after exposure to food or allergen.
• severe allergic reactions termed anaphylaxis can also present with laryngeal edema and angioedema

2. Hereditary angiodema

• Non inflammatory swelling typically involves the face, lips and genitalia. Also involves the gastrointestinal and genitourinary tract.
• Several types of HAE have been identified. Type 1 is associated with the deficiency and type 2 with the destruction of the C1 inhibitor protein resulting in deranged complement levels. Type 3 is associated with normal C1 inhibitor and complement levels.
• pathophysiology of HEA that involves the destruction, defect or deficiency of C1 inhibitor allowing for the accumulation of excessive amounts of bradykinin, a peptide responsible for promoting inflammation and leakage of excessive fluid from the walls of blood vessels into the tissues.
• Autosomal dominant pattern of inheritance usually showing at least one family member presenting with similar signs and symptoms typically in late childhood or adulthood
• Episodes are usually triggered by infection or underlying trauma.

3. Acquired angiodema

• ACE inhibitors are the most common cause of acquired angiodema
• pathophysiology involves inhibition of angiotensin converting inhibitor that predisposes to the accumulation of bradykinin.
• presentation within days to weeks after initiation of ACE Inhibitors and commonly involves the larynx, glottis, mouth and lips.

DIAGNOSIS

• Diagnosis of angiodema is usually made clinically

• HEA can be confirmed by measuring the complement levels and C1q levels. Genetic testing for mutations can also be done in certain cases.

MANAGEMENT

• Angiodema due to any cause associated with airway compromise and vasomotor instability requires treatment with IM epinephrine and endotracheal intubation in cases of severe laryngeal edema
• Allergen associated angiodema requires the discontinuation of the offending agent
• Acute attacks of type 1 and 2 HEA can be benefited with the use of C1 inhibitor concentrates and a kallikrein inhibitor
• Acquired angiodema requires the discontinuation of ACE inhibitors

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