The Journal of Dermatology
Vol. 26: 532-534, 1999

Short reports

Sneddon's Syndrome and Antithrombin III
Ertugrul Bolayir, Hulusi Kececi, Melih Akyol*,
Aysenur Tas and Murat Polat*
Abstract

Sneddon's syndrome, an uncommon disorder, is characterized by multiple cerebrovascular accidents along with ideopathic livedo reticularis. In this article, two cases who had
increased amounts of antithrombin III and were diagnosed with Sneddon's syndrome are
presented.

Key words: Sneddon's syndrome; livedo reticularis; cerebrovascular accident;
antithrombin III

Case Report
Case 1:
A 30-year-old woman who had a history of
epileptic seizures was admitted to the Department of Neurology with complaints of dysarthria
and hemiparesis. There was no family history.
Neurologic examination revealed left hemiparesis, dysarthria, and central facial paresia. Dermatologic examination revealed reticular erythema
on the extremities (Fig. 1). Routine hematologic
and urine analyses were in their normal ranges.
Echocardiography, electrocardiography, cerebral angiography, carotid ultrasonography, and
posteroanterior chest X-ray were normal. Computed tomography (CT) demonstrated multiple
infarcts (Fig. 2). Anticardiolipin antibodies, protein C, and protein S were in their normal
ranges. Antithrombin III (ATIH) was 45 mg/dl
(normal range: 22-39 mg/dl).
Case 2:
A 31-year-old woman who had epileptic
seizures and unconsciousness was admitted to
the emergency service. Neurologic examination
revealed unconsciousness and right hemiplegia.
Dermatologic examination revealed reticular
Received February 17, 1999; accepted for publication April 21, 1999.
Department of Neurology and *Dermatology,
Medical Faculty, Cumhuriyet University, Sivas, Turkey.
Reprint requests to: Ertugrul Bolayir, Department
of Neurology, Medical Faculty, Cumhuriyet University, 58140-Sivas, Turkey.

Fig. 1. Reticular erythema on the lower extremities.
erythema on the upper extremities (Fig. 3).
Routine hematologic and urine analyses were in
that normal ranges. Echocardiography, electrocardiography, cerebral angiography, carotid ul-

Sneddon's Syndrome and Antithrombin III

533

Fig. 3. Reticular erythema on the upper extremities

Fig. 2. CT showing multiple infarct areas

Fig. 4. CT showing infarct in the left hemisphere

trasonography, and posteroanterior chest X-ray
graphy were normal. CT demonstrated acute infarcts of the areas supplied by the middle cerebral arteries (Fig. 4). Anticardiolipin antibodies,
protein C, and protein S were in their normal
ranges. ATIII was 60 mg/dl (normal range:
22-39 mg/dl).

Discussion
Sneddon's syndrome, characterized by idiopathic livedo reticularis and cerebrovascular diseases, is a rare disorder. There may be
both arterial and venous obliteration and visual disorders, headache, and ischemic encephalopathy associated with this disorder
(1,2). Sneddon's syndrome usually occurs
in association with antiphospholipid antibodies (2,3).
In most cases, early symptoms occur in
the second and third decades. The incidence of Sneddon's syndrome is greater in
females than males. Before the cerebrovascular problems arise variable forms of
epilepsy can be seen (4, 5). In our cases, the
early symptoms were epileptic seizures.
The cerebrovascular pathology includes
transient ischemic attacks and strokes. Cerebral infarcts usually are found in the areas
supplied by the middle diameter arteries.
Hypertension and memory impairment can
also be associated with Sneddon's syndrome
(6-8). CT reveals cortical atrophy in variable degrees and isolated or multiple infarcts (4). In our cases, CT demonstrated
multiple infarct areas.
Antiphospholipid antibodies may playa
role in the pathogenesis of Sneddon's syndrome (3). However, some cases may not
have antiphospholipid antibodies (9).
These antibodies are also found in about

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Bolayir et al

1-2% of the normal population, although
they are found in about 30% of the patients
with Sneddon's syndrome. Aznar et al. detected antiphospholipid antibodies only in
one of seven cases. Therefore, in addition to
antiphospholipid antibodies, other factors
must be involved in the pathogenesis of
Sneddon's syndrome (10).
We found that our cases have high levels
of ATIII, a plasma protein that inactivates
serine proteases by an irreversible time-dependent reaction, which is often designated
as a progressive inhibitor. It has been purified extensively and has been separated
from other progressive antithrombins.
ATtlI is a plasma glycoprotein that increases
its ability to inhibit thrombin and other activated clothing factors. In several variant
forms of the disorder, plasma levels of ATIII
antigen are normal, but those of functional
ATIlt are depressed (11-14). Akiguchi and
co-workers (15) reported a family with antiphospholipid antibody syndrome as well as
Binswanger's disease with increased coagulation activity. In one patient from this family, abnormally high levels of thrombin-antithrombin complex and fibrinogen were
detected. Moreover, Akiguchi and co-workers (16) reported another family in which
several members had a history of subcortical
vascular dementia and coagulation abnormalities. One of the members of this family
had Sneddon's syndrome and abnormally
high thrombin-antithrombin complexes.
The patient improved both clinically and biologically when treated with antithrombin.
A qualitative deficiency of ATIII may explain why ATIII levels are high in our cases.
We think that a qualitative deficiency of
ATIII will prove to playa role in the pathogenesis of Sneddon's syndrome.
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