Case Report
Localized Cold Urticaria Mimicking Livedo in an Adult Female
Keiji Sugiura* and Mariko Sugiura
Department of Environmental Dermatology and Allergology, Daiichi Clinic, Japan
*Corresponding author: Keiji Sugiura, Department of Environmental Dermatology and Allergology, Daiichi Clinic, Nittochi Nagoya Bld. 2F, 1-1 Sakae 2, Nakaku, Nagoya, 468-0008, Japan
Published: 28 Oct, 2016
Cite this article as: Sugiura K, Sugiura M. Localized Cold
Urticaria Mimicking Livedo in an Adult
Female. Ann Clin Case Rep. 2016; 1:
1168.
Abstract
There are few reports of localized urticaria with symptoms to similar to livedo racemosa (reticularis) in adults. Here we describe a rare case of localized cold urticaria (grade 1) on the face of a 32-yearfemale. The results of an ice cube test showed a positive reaction on the affected area (her cheeks). Treatment with anti-histamine (second generation) tablets, warming and avoidance of cold exposure were the best treatments for her cold urticaria in winter.
Keywords: Primary biliary cirrhosis; Terlipressin; Intestinal ischeamia; Variceal bleeding; Vasopressin
Introduction
Cold urticaria is caused by cold exposure, and localized cold urticaria in adults is rare. Cold
urticaria is divided into three grades: grade 1 is localized urticaria; grade 2 is urticaria or angioedema
without respiratory symptoms or hypotension; and grade 3 is severe, systemic allergic reactions
(gastrointestinal, cardiovascular, respiratory symptoms or shock) [1]. Generally, cold urticaria
reaches grade 2 severity. Acquired cold urticaria is predominantly shown in young adults [2].
Livedo shows a reticular cutaneous pattern, and livedo are divided to three types: cutis marmorata,
livedo reticularis and livedo racemosa. Most livedo reticularis and racemosa cases are caused
by disturbances of the peripheral circulation of underlying disease with pain on the legs. Cutis
marmorata is developed by physiological circulation in younger people than livedo reticularis and
racemosa.
Here we describe a rare case of localized cold urticaria (grade 1) similar to livedo racemosa
(reticularis) on the face of an adult female. We previously reported an adult male case of localized
cold urticaria [3]. This case, like our previous one, developed wheals (a reticular pattern) only in winter and without any underlying disease; in both cases, the cutaneous lesions were caused by cold
environmental factors (wind, snow, rain and cold temperatures).
Case Presentation
A 32-year-female reported developing reticular cutaneous pattern lesions with whealand
discomfort (slightly itching) on her cheeks every winter (Figure 1). Lesions had developed on her face, and her skin condition improved when she took anti-histamine tablets, warmed herself and
avoided cold exposure.
Our first diagnosis was livedo or localized cold urticaria. She had no pain, dyspnea, diarrhea,
cutaneous ulcers or any wheal on any part of her body other than her face when she was exposed
to cold. She had no history of any other disease (bacterial or virus infection, collagen disease, heart
disease, vasculitis, rheumatoid arthritis, amyloidosis or sarcoidosis). There was no familial history
of cold urticaria. Laboratory data were in the normal range. She did not have cryoglobulins or cold
hemolysins. The results of an ice cube test showed a positive reaction on the affected area (her
cheeks). She did not consent to a skin biopsy, but treatment with anti-histamine (second generation)
tablets, warming herself and avoiding cold exposure improved her cutaneous condition. We made a
final diagnosis of localized urticaria based on her symptoms.
Discussion
Urticaria occurs in about 15-20% of the population [4], and the frequency of cold urticaria
is in 2‒6% of urticaria patients [5-7]. There are few reports of localized urticaria in adults. The immunologic and non-immunologic mechanisms of cold urticaria are unclear, but some chemical
mediators might be involved [8,9]. The mechanisms by which chemical mediators are activated in
a local area might be similar to the mechanisms of allergic contact urticaria. Specific skin antigens activated in a cold environment might release pro-inflammatory
mast cell mediators and histamines [1,10-13]. Not only immunologic
but also non-immunologic reactions could be associated with the
mechanisms of localized urticaria; for instance, local vasoactive
mediators could be activated by cold exposure.
Generally, acquired and generalized cold urticaria can be easily
diagnosed based on the episodes. Some cases of localized cold
urticaria are not as easy to diagnose because the skin lesions look like
other dermatoses (insect bites, irritant dermatitis, contact urticaria,
and livedo). This case presented in a pattern similar to that of reticular
racemosa, making differential diagnosis important. There is a check
list for cold urticaria [14] that is useful for the diagnosis of this
condition.
The initial and essential treatment of localized urticaria is to
warm the whole body or affected area and avoid cold exposure. The
second treatment is the administration of antihistamine tablets or
leukotriene antagonists. Previous data showed that up-dosing of the
antihistamine is significantly more effective for reducing symptoms
than administering the standard dose [15,16]. We administered
antihistamine tablets at the standard dose in our two cases, but
the patients developed localized urticaria nonetheless. Because
their localized urticaria was not inhibited by the standard dose of
antihistamine tablets, we speculated that these cases resulted from
a dose deficiency of the medication or other causative factors of
localized urticaria.
Figure 1
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