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Acquired Angioneurotic Edema (AAE) is characterized by profound swelling of the dermis associated with abdominal pain. Edema is usually painless, nonpruritic, nonurticarial, and nonpitting. Edema of the larynx and other portions of the airways is the most fearsome feature of this disorder and can be life-threatening.

Acquired Angioneurotic Edema (AAE); Bannister Disease/Syndrome; Milton Urticaria; Milton-Quincke Syndrome; Quincke Disease/Syndrome.

The German internist Heinrich Iranaeus Quincke (1842-1922) was by no means the first to report the disease. This honor goes to the Italian physician Marcello Donati, who described a young count with sensitivity to eggs. Quincke however was the first to publish a review (without referencing earlier descriptions of the disease) in 1882.

Varies with etiology; hereditary angioneurotic edema (HAE) is found in 1:10,000 to 1:150,000 persons. AAE has been reported in fewer than 50 cases. Angioedema following use of angiotensin-converting enzyme (ACE) inhibitors is found in 1 to 2 cases per 1000 persons. Other forms of angioedema (not secondary to HAE or AAE) will affect 10 to 20% of the population at some time in their lives.

Angioedema is associated with the following disorders: 1) acquired C1-esterase inhibitor deficiency and 2) hereditory C1-esterase inhibitor deficiency. Traditionally two types of HAE have been described. HAE type I, which accounts for approximately 85% of patients is the result of decreased C1-esterase inhibitor production. The remainder 15% of patients suffer from HAE type II, which is characterized by normal or even elevated levels of a functionally impaired C1-esterase inhibitor. (An HAE type III has recently been assigned to a possibly X-linked inherited form of HAE.) ACE-induced angioedema is idiopathic.

Allergic (IgE-mediated) may be caused by inhalants (ethylene-glycol), bites and stings, drugs (morphine, codeine, iodine-dye, aspirin, or nonsteroidal anti-inflammatory drugs) and sera, foods and food additives, physical induction (cold, exercise, pressure, vibration, facial piercing), parasitic infections, paraneoplastic or autoimmune disorders.

The hereditary form is autosomal dominant transmitted and caused by a mutation in the C1 inhibitor gene (C1-esterase inhibitor) located on chromosome 11p11.2-q13.1. Male:female ratio is 0.85, but 20% are de novo mutations. The acquired form is often associated with B-cell lymphoproliferative disorders.

C1, the first component of the classical pathway of the complement system is a complex macromolecule. Activation leads to cleavage producing the C1 esterase, which may now act on C4 and C2. C1-esterase inhibitor is the control protein that inhibits the spontaneous activation. It is also able to inhibit the Hageman factor (XIIa), plasma thromboplastin (XIa), and kallikrein.

Clinical (mildly or nonpruritic, relapsing edema that does not respond to antiallergic treatment). Diagnosis of hereditary form is difficult; precise criteria have been validated by the European study group on HAE (“Oedème Angioneurotique”, OAN). One major clinical sign is either a) limited subcutaneous angioedema, not associated with urticaria, lasting at least 12 hours and relapsing frequently; or b) recurrent idiopathic abdominal pain lasting at ...

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