Clinical Brief
 By Michelle Greer, RN, IgCN
HUNDREDS OF conditions are
suspected or confirmed to be
autoimmune conditions, while other
conditions can be caused by various
factors, including a malfunction in the
immune system. Urticaria, caused by
the latter, is a skin condition resulting
in a rash. The rash can range from mild
to severe, with red welts or hives and
pruritus (itching) that can be mild or
intense. Dangerous swelling can also
occur. In addition to a rash, which
can occur anywhere on the body,
headache, dizziness, hoarseness of voice,
shortness of breath, nausea, vomiting
and abdominal pain may occur as
concomitant systemic manifestations of
severe episodes of urticaria.1
Overall, urticaria occurs frequently
in the U.S. population, with nearly one
in four persons experiencing it at some
point in life. The subtypes of urticaria
are inducible (expressed in response to
a stimulus) and spontaneous (expressed
without external stimulus), which
may be acute or chronic.2 Chronic
spontaneous urticaria (CSU) can
represent the leading sign of a wide
spectrum of systemic diseases, including
primary immunodeficiencies.3
Causes of Urticaria
Urticaria can be caused by several
factors (Figure 1), including:
• an allergic reaction to food or
medication • an environmental trigger such as
pollen or dust
• an infection
• psychological issues or stress
• an unknown cause, known as
idiopathic • autoimmune, known as chronic
autoimmune or CSU
• physical causes such as:1
– dermatographism/dermographism
(the development of red, swollen lines
or welts, called wheals, on the skin that
occur with pressure, firm stroking or
light scratching)
Figure 1. Causes of Urticaria1
Physical 35%
Idiopathic/Others 30%
Autoimmune 25%
Vasculitic 5%
Pseudoallergic 3%
Infectious 2%
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– delayed
pressure urticaria
(recurrent erythematous and often
painful swelling after the skin is exposed
to sustained pressure)
– cold urticaria (a skin reaction to
cold that appears within minutes after
cold exposure)
– aquagenic urticaria (a rare
condition in which hives develop
rapidly after the skin comes in contact
with water, regardless of its temperature)
– cholinergic urticaria (a reaction
that results in tiny hives surrounded by
large patches of red skin related to an
increase in body temperature)
– solar urticaria (a rare allergic
reaction to sun exposure)
– vibratory urticaria (hives, swelling,
redness and itching caused by exposing
the skin to vibration, repetitive
stretching or friction)
Diagnosing Urticaria
Various specialists can diagnose
urticaria, including internal medicine
physicians, immunologists
and dermatologists. For episodes of
inducible urticaria, the causative factor
should be identified and eliminated, but
some types such as solar urticaria are not
curable. In acute cases of spontaneous
urticaria, the rash responds to treatment
and resolves. However, some cases can
become chronic, which is defined as
lasting six weeks or longer. The causative
factor of CSU is difficult to identify, and
most of the time it remains unknown.

Chronic autoimmune urticaria, a form
of CSU, can be extremely debilitating
and difficult to treat. It can be diagnosed
with a positive blood test from a chronic
urticaria (CU) index panel, which
looks for the presence of histamine-



Clinical Brief
Figure 2. Step-Down Treatment for Chronic Urticaria
globulin (IVIG) may be beneficial, with
several case reports and other studies
showing efficacy.

In one study of three patients, all
showed a response to IVIG, with the
researchers concluding that IVIG
remains a viable alternative in patients
with persistent urticaria with evidence
of increased basophil CD203c
expression unresponsive to first-line
therapies.5 Another study concluded
high-dose IVIG represents an important
therapeutic option in patients with
severe CSU.2
Research Provides Hope
In summary, there are many types of
urticaria with many causes, as well as
a variety of treatment options for both
acute and chronic cases. There are also
many active clinical trials exploring
various medications and treatments for
chronic urticaria that are providing hope
for controlling this debilitating illness.

 Source: Fine, LM, and Bernstein, JA. Guideline of Chronic Urticaria and Beyond. Allergy, Asthma and Immunology Research, 2016 Sep; 8(5): 396–403. Accessed
at www.ncbi.nlm.nih.gov/pmc/articles/PMC4921693.

releasing autoantibodies, signifying
autoimmune involvement. A few
different autoantibodies cause chronic
autoimmune urticaria, and while the
CU index panel does not identify which
autoantibody is present, it does provide
the needed information to prescribe a
proper treatment plan. Studies with a CU
index panel performed at the University
of Iowa indicated 41 percent of chronic
autoimmune urticaria patients were
positive for this autoantibody, which is
similar to the prevalence in published
studies.4 Therefore, even though CSU
affects only approximately one percent
of the population, in 41 percent of those
cases, a histamine-releasing autoantibody
can be identified with this laboratory test.

Treating Urticaria
Treatment of urticaria depends on the
cause and type. Treatment of chronic
urticaria may include various types of
oral, topical or eye drop antihistamines,
steroids and immunosuppressants. In
general, urticaria can be successfully
treated using one or more of these
medications (Figure 2). For chronic
autoimmune cases resistant to these
treatments, intravenous
immune 1. Sachdeva, S, Gupta, V, Amin, SS, and Tahseen, M. Chronic Urticaria. Indian
Journal of Dermatology, 2011 Nov;56(6):622-8. Accessed at www.ncbi.nlm.

nih.gov/pmc/articles/PMC3276885. 2. Mitzel-Kaoukhov, H, Staubach, P, and Müller-Brenne, T. Effect of High-
Dose Intravenous Immunoglobulin Treatment in Therapy-Resistant Chronic
Spontaneous Urticaria. Annals of Allergy, Asthma and Immunology, 2010
Mar;104:253–8. Accessed at www.pubmed.ncbi.nlm.nih.gov/20377115.

3. Comberiati, P, Costagliola, G, Carli, N, et al. Refractory Chronic Spontaneous
Urticaria Treated with Omalizumab in an Adolescent with Common Variable
Immunodeficiency. Frontiers in Immunology, 2019 Jul 17; 10:1700. Accessed
at www.ncbi.nlm.nih.gov/pmc/articles/PMC6652742.

4. The University of Iowa Department of Pathology. Chronic Urticaria (CU)
Index Panel. Accessed at www.healthcare.uiowa.edu/path_handbook/
handbook/test2886.html. 5. Amar, SM, Harbeck, RJ, and Dreskin, SC. Effect of Intravenous
Immunoglobulin in Chronic Urticaria with Increased Basophil CD203c
Expression. The Journal of Allergy and Clinical Immunology, Vol 121, Issue
2, Supplement 1, S98. Feb. 1, 2008. Accessed at www.jacionline.org/article/
S0091-6749(07)02817-5/fulltext. MICHELLE GREER, RN,
IgCN, is senior vice president
of sales at Nufactor, a specialty
infusion company.

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| December-January 2023
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