ARTICLE
Auteur(s) : Yu SAWADA, Toshinori BITO, Rieko KABASHIMA,
Kazunari SUGITA, Motonobu NAKAMURA, Yoshiki TOKURA long-ago@med.uoeh-u.ac.jp
Department of Dermatology, University of Occupational and
Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu,
Japan
Cholinergic urticaria (CU) is a rare condition clinically
characterized by pinpoint-sized, highly pruritic wheals, a unique
urticaria which is typically provoked by exercise, warmth, and
emotional distress [1]. CU is occasionally associated with
depressed sweating, known as hypohidrosis (incomplete lack of
sweating) or anhidrosis (complete lack of sweating) [2]. 29
patients have been reported with CU with hypohidrosis and/or
anhidrosis (CUHA) [2], and 26 are Japanese. Rarely, CU patients
have episodes of seizures upon occurrence of urticaria, with or
without abnormalities in electroencephalography [3, 4]. We report a
case of CUHA, manifesting with epileptic seizures.
A 23-year-old man presented a 1-year history of wheal,
generalized hypohidrosis, and heat intolerance, with pain in a hot
environment. He was admitted to our hospital for evaluation of his
skin eruption. The skin surface was divided into two areas,
anhidrotic and hypohidrotic, by iodine-starch reaction. The
hypohidrotic areas included the face, chest, abdomen and arms
(figure
1A), and anhidrosis was seen on the back, buttocks
and legs (figure 1B),
when compared to a sex and age-matched healthy control. On
exercise, he developed wheals, which coincided with sweat orifices
at the hypohidrotic areas (figure 1C),
but no wheals were seen in the anhidrotic areas. Autologous sweat
and serum tests were negative. Acethylcholine injection yielded a
wheal reaction at a hypohidrotic but not an anhidrotic area. A skin
biopsy taken from an anhidrotic area revealed lymphocytic
infiltration around the sweat glands (figure 1D),
but no infiltration was observed in the hypohidrotic area. Skin
sections were immunohistochemically stained with anti-cholinergic
receptor muscarin 3 (CHRM3) antibody (H-210, 1:50; Santa Cruz
Biotechnology, Santa Cruz, CA) [2], which is the most important
cholinergic receptor (CHR) for sweating. The expression of CHRM3
was reduced in the eccrine glands of the hypohidrotic area
(figure
1E) and was not absent in the anhidrotic skin
(figure
1F) compared to a healthy control (figure 1G).
We diagnosed the disorder as CUHA. Since anti-histaminic drugs
showed no therapeutic effect, we gave this patient
methylprednisolone pulse therapy (1 g daily for 3 consecutive
days). Five days after the treatment, when his sweating began to
recover, the patient had a seizure, he suddenly twitched and shook
his limbs, and his eyes rolled back. The seizure lasted 2 minutes.
He also had several episodes of the same symptoms when sweating
with exercise. There was no abnormal finding in the blood levels of
glucose, sodium, electrolytes or in brain computed tomography.
Neurological examination was normal. After his sweat condition was
substantially improved, epileptic seizures did not occur.
Although little is known about the exact prevalence of CUHA in
the general population, the incidence of CU is 11.2%, mostly in
younger people [5]. The 1-year prevalence for epilepsy is 7.1/1000
[6]. Therefore, it is considered that the two diseases are not a
coincidence in our case. Our review of the English and Japanese
literature found three cases of epileptic seizures associated with
CU, including our case. All cases were young Japanese. When the
intensity of sweat-promoting stimuli is high, the autonomic center
in the diencephalon or brain stem is abnormally activated [3].
CHRM3 expression is decreased in patients with CUHA, and steroid
therapy might recover the CHR expression in sweat glands [2]. The
decreased expression of CHR was re-expressed after the steroid
pulse therapy, not only in the sweat glands but also in the brain,
and acethylcholine might highly stimulate the autonomic center,
resulting in an epileptic seizure.
Disclosure
Financial support: none. Conflict of interest: none.
References
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