Angioedema by.. Kunkanit Suntipraron

Angioedema swellings occur deeper in the dermis and in the subcutaneous or submucosal tissue. They may also affect the face,mouth,tongue and rarely in bowel. angioedema occurs in deep skin layers where there are fewer mast cells and sensory nerve ending,the lesions have little or no associated pruritus and the swelling may be described as painful or burning Hereditary angioedema has AD inheritance pattern,occurs in approximately 1:50,000

Pathogenesis

MAST CELLS (CUTANEOUS)

complement system

Hageman factor-dependent

Text

HD type I,II Types I and II are caused by mutations in one allele of the structural gene for C1 inh, resulting in reduced levels of C1 inh (85% of cases; type I) or reduced C1 inh function (15% of cases; type II). facial,laryngeal edema or colicky abdominal pain. Trauma (emotional or physical) and estrogens can trigger attacks. The usual duration is 48–72 hours.

HD type III Type III HAE refers to individuals with a heterozygous gain of function mutation in the gene that encodes FXII. Compared to patients with an inherited deficiency of C1 inh, the age of onset is later (usually in the second decade) and there is a higher frequency of facial angioedema.

acquired HD lymphoma ; IgM connective tissue disease cryoglobulinemia response to androgen therapy

ACEI and angioedema

TReatment protect airway Epinephrine; indicate laryngeal edema corticosteroid antipruritus antihistamine Leukotriene antagonist

case..

A 19-year-old woman presents to the emergency department with light headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier. The patient reports previous episodes of abdominal pain and swelling of her hands and feet that have been attributed possibly to food allergies, which have recently become more frequent. There is no associated urticaria. Her only medication is an oral contraceptive that was started 3 months earlier. She notes a history of similar episodes in her father. She is afebrile, with a blood pressure of 75/40 mm Hg, a pulse of 120 beats per minute, and diffuse abdominal tenderness with guarding and rebound tenderness. How should her case be evaluated and treated?

- low antigenic C4 level - normal antigenic C1 and C3 levels. ( Measurement of C4 levels is a cost effective screening test to rule out hereditary angioedema) - the C4 level is normal between attacks. Subsequent measurement of antigenic and functional C1-inhibitor levels confirms the diagnosis of hereditary angioedema and distinguishes between type I (low antigenic and functional C1-inhibitor levels) and type II (normal antigenic C1-inhibitor level but low functional C1-inhibitor activity)

Hereditary angioedema type II

Short-Term Treatment ; laryngeal angioedema,who may require emergency intubation if the swelling worsens. Purified C1-inhibitor replacement therapy Neither corticosteroids nor antihistamines have been shown to provide a meaningful benefit during attacks of hereditary angioedema and Long term prophylaxis ;

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