20
Cutaneous Attacks
As stated earlier, cutaneous attacks of HAE involve non-
pruritic swelling of the skin. Because small vessels in deep
subcutaneous tissue are usually involved, the swollen areas
usually have indistinct borders and do not resemble the hives
of allergic reaction that are mediated by immunoglobulin E
and induce histamine production.
5
Therefore, HAE attacks
are not relieved by antihistamines or corticosteroids.
5,8,11
Although cutaneous edema can be present anywhere in
the body, the most common sites of cutaneous angioedema
are, the extremities (hands, arms, feet, and legs).
5,31
As
shown in Table 4, the face and genitals can also be sites of
angioedema, but account for a much smaller percentage of
all attacks.
5,31
Bork et al found that genital swelling tended
to last about 3 days.
31
It should be noted that perioral
angioedema is not typical of HAE, but is common in other
etiologies of angioedema.
5
Most often, angioedema occurs in a single site, expands
over 12 to 24 hours, and then dissipates over the next
2 to 5 days. Facial edema typically persists longer than
angioedema in other areas of the body.
35
The angioedema of HAE is not histamine-
mediated and, unlike angioedema of other
etiologies, will not respond dramatically to
antihistamines or corticosteroids.
Table 4 – Per-episode and Per-patient Analysis of
Cutaneous Swelling Sites
31
Figure 9 – Computed Tomograms of the Abdomen,
Showing Perihepatic Effusion (Top) and Intestinal
Wall Edema (Bottom) During an Attack of Angio-
edema in the Gastrointestinal Tract
Number (%)
of Attacks
n=131,110
Number (%)
of Patients
a
n=209
Type of Attack
65,102 (50%)
59,095 (45%)
2,134 (2%)
2,741 (2%)
1,132 (1%)
201 (96%)
196 (94%)
158 (76%)
131 (63%)
57 (27%)
All cutaneous
Site of Cutaneous Attack
Extremities
Face
Genitals
Trunk, neck
a
209 of 221 patients were symptomatic; 201 had cutaneous swelling.
Picture was provided courtesy of Bork et al.
37
Abdominal Attacks
Abdominal attacks are characterized by mild to severe
spasmodic pain and cramping. These episodes can be
associated with dizziness, nausea, vomiting, and diarrhea.
Leukocytosis and ascites may also occur.
5
Signs and
symptoms of an abdominal attack can mimic those of acute
abdomen. As a result, about one third of individuals with
HAE endure unnecessary GI surgeries.
5,30
The attacks are
usually preceded by extraabdominal symptoms. In many
patients, the periodic abdominal attacks may precede
attacks of the extremities, contributing to a delay in dia-
gnosis.
36
Edema of the GI wall can be a distinguishing feature; it is
rarely seen in angioedema of other origins (Figure 9).
