QUINCKE’S
SYNDROME
By: KRANJ GOSAI
432-A
DEFINITION
Angioedema or Quincke's edema is the rapid swelling
(edema) of the dermis, subcutaneous tissue, mucosa and
submucosal tissues.
It is very similar to urticaria, but urticaria, commonly
known as hives, occurs in the upper dermis.
TYPE
1. Acute or allergic angioedema - the swelling is caused by an
allergic reaction, such as a reaction to peanuts.
2 Idiopathic. Angioedema – there is no known cause for the
symptoms of swelling.
3. drug-inducedangioedema - the swelling is a side effect of certain
medications.
4. hereditaryangioedema the swelling is caused by 'faulty' genes
that are inherited from a person's parents.
OR ANGIOEDEMA IS
CLASSIFIED AS EITHER
HEREDITARY
1. Acquired angioedema
OR ACQUIRED
can be immunologic, nonimmunologic, or idiopathic.
It is usually caused by allergy and occurs together with other
allergic symptoms and urticaria.
It can also occur as a side effect to certain medications, particularly
ACE inhibitors.
HEREDITARY ANGIOEDEMA
(HAE):
exists in three forms, all of which are caused by a genetic
mutation inherited in an autosomal dominant form.
They are distinguished by the underlying genetic
abnormality.
All forms of HAE lead to abnormal
activationof the complement system, and
all forms cancause swelling elsewhere in
the body, such asthe digestive tract.
DRUG ALLERGY
Medicines (drug allergy), such as antibiotics (penicillin),
nonsteroidal anti-inflammatory drugs (NSAIDS), and
blood pressure medicines.
WHEN THEDRUG ALLERGY Pollen infections or with
other illnesses (including autoimmune, and leukemia and
lymphoma).
Family history (HAE)
PATHOPHYSIOLOGY
Angioedema is swelling due to increased vascular permeability and
extravasation of intravascular fluid.
Known mediators of increased vascular permeability include the
following : Mast cell-derived mediators (e.g. histamine).
2. Bradykinin, a peptide containing nine amino acid residues
produced from a2-globulin by the enzyme kallikrein.
Bradykinin is a potent vasodilator, and complement-derived
mediators.
HEREDITARY ANGIOEDEMA
Hereditary angioedema (HAE) is due to mutations within the C1-
INH gene and is transmitted as an autosomal dominant trait.
C1INH is important in regulating the classical complement system
and contact cascade. • Respectively, Insufficient C1INH function
leads to dysregulation of each of these pathways.
However, contact pathway dysregulation is largely responsible for
the symptoms of HAE via excess generation of bradykinin