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
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
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
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 ...