Lupus (2000) 9, 301±303
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CASE REPORT

Red ear(s) syndrome associated with child neuropsychiatric
systemic lupus erythematosus
1

H-S Lee1, S-C Bae1*, W-S Uhm1, J-B Jun1, I-H Lee1 and SY Kim1

The Hospital for Rheumatic Diseases, Department of Internal Medicine, Hanyang University College of Medicine, Seoul, South Korea

This case illustrates that a child having severe neuropsychiatric systemic lupus erythematosus
(NPSLE) with seizure and cerebral vascular disease showed excellent clinical outcome in response
to intravenous methylprednisolone and cyclophosphamide pulse, and presented unexplained red
ears phenomenon.
Lupus (2000) 9, 301±303
Keywords: systemic lupus erythematosus; neuropsychiatric systemic lupus erythematosus; seizure;
cerebral vascular disease; red ear(s) syndrome

Introduction

Case report

The prevalence of neuropsychiatric systemic lupus
erythematosus (NPSLE) in children has varied,
occuring in 9 ± 45% of children with SLE (systemic
lupus erythematosus).1 ± 3 Recently there were a few
reports of the clinical manifestation, therapy, or
prognosis of NPSLE in childhood.1 ± 5 The effect of
a combined treatment of intravenous methylpredni
solone (IVMP) and intravenous cyclophosphamide
(IVCy) in children with severe NPSLE was observed
to be favorable.4
Red ear(s) syndrome was ®rst described by Lance.6
He suggested that many etiologies, such as irritation
of the third cervical root, temporomandibular joint
dysfunction or thalamic syndrome, could be responsible for `red ear(s) syndrome'. Hirsch also reported
red ear(s) syndrome among patients with headache.7
To our knowledge, however, there has not been any
report of red ear(s) syndrome in SLE patients.
We report a case of severe NPSLE (seizure and
cerebral vascular disease) with unexplained red ear(s)
syndrome.

A 9-year-old patient, Korean ± Swiss, visited the
Emergency Room due to partial-complex seizure with
partial impairment of consciousness, which lasted for
about 5 min. Three months prior to admission, he had
already been diagnosed as having SLE based on
typical malar rash, positive ANA, leukopenia and
positive anti-dsDNA. But there had not been any
history of arterial or venous thrombosis, and livedo
reticularis. He had been placed on prednisolone 30 mg
and hydroxychloroquine 150 mg daily. Laboratory
®ndings at admission were as follows; 2400=mm3 of
WBC, 9.6 g=dl of hemoglobin, 2626103=mm3 of
platelets, normocytic normochromic anemia and
leukopenia without schistocytes on peripheral blood
smear, normal urinalysis, 218 mg=dl of cholesterol,
95 mg=dl of glucose, 24=28 units of ALT=AST, 280
units of LDH, 0.7 mg=dl of creatinine, normal
electrolytes, 0.40 mg=dl of C-reactive protein,
58 mm=h of ESR, 16.6=4.02 mg=dl of C3 and C4,
positive ANA test (speckled type of 1:320 and
cytoplasmic type of 1:160), negative anti-dsDNA
(using Crithidia lucillae), positive anti-Ro antibody,
negative antiribosomal P protein, non-reactive VDRL,
negative anticardiolipin antibody and lupus anticoagulant, weakly positive direct Coombs' test. MRI
showed the infarction of left basal ganglia and
prominent vasculitis of left middle cerebral artery
(Figure 1(a)).

*Correspondence: S-C Bae, The Hospital for Rheumatic Diseases,
Hanyang University Medical Center, Seoul 133-792,
South Korea.
Tel: ( ‡ 82) 2 2290 9203 or 9246; Fax: ( ‡ 82) 2 2298 8231;
E-mail: scbae@email.hanyang.ac.kr
Received 1 September 1999; accepted in revised form
23 November 1999

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Red ear(s) syndrome in SLE
H-S Lee et al

302

On the second day, another attack of seizure,
altered mentality, and right hemiplegia developed.
According to ACR nomenclature and case de®nitions
for NPSLE,8 he had seizure disorders and stroke
syndrome as manifestations of NPSLE. After intravenous bolus injection of diazepam, we tried intravenous methylprednisolone 500 mg pulse (IVMP) and
intravenous phenytoin loading. Fifteen minutes after
IVMP, right hemiplegia recovered completely and his
mentality was cleared. Two additional IVMP daily in
succession and intravenous cyclophosphamide 400 mg
pulse (IVCy) were done. He started on oral prednisolone 40 mg, oral phenytoin and hydroxychloroquine 200 mg daily.
Brain perfusion SPECT (single photon emission
computed tomography) was done, which showed
decreased perfusion of left basal ganglia (right=left
perfusion ratio of basal ganglia was 1.32). EEG did
not show any abnormal ®ndings.

Figure 1(a) T2 weighted MRI of brain showed diffuse brain atrophy,
high signal intensity of left basal ganglia, multifocal high signal spots of
subcortical white matter and prominent vascularity of left middle cerebral
artery. (b) Follow-up T2 weighted MRI showed that the volume of high
signal intensity of left basal ganglia got much smaller without enhancement but a small infarction area at the left putamen still remained.
Previous vasculitic lesion of left MCA artery territory disappeared
completely but multifocal high signal spots were persistent.

On the 30th hospital day, the second cycle of IVMP
500 mg and IVCy 500 mg was given. On the 37th
hospital day, follow-up brain MRI showed that the
infarcted area at the left basal ganglia got much
smaller but still remained, and previous vasculitis of
left MCA artery territory disappeared completely
(Figure 1(b)).
Follow-up laboratory ®ndings were as follows;
5600=mm3 of WBC, 11.4 g=dl of hemoglobin,
3116103=mm3 of platelet. His complements were
becoming stabilized from 16.6=4.02 mg=dl to 80.9=
12.3 mg=dl of C3=C4.
Despite stable and good clinical condition, he had
suffered episodes of reddish swelling of the helices of
the ears, accompanied by a mild burning sensation of
auricle, mild headache, local heat, tinnitus, and pain
of mastoid area (Figure 2). This usually occurred
bilaterally, once or twice each day, lasted for about
one hour, and was more common in the evening.
There were no precipitating factors such as fever,

Figure 2 Right ear helix showed redness accompanied with mild burning
and local heat.

Lupus
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Red ear(s) syndrome in SLE
H-S Lee et al

touching, chewing, grinding, or neck pain. Radiographs of the mastoid bone and paranasal sinuses,
audiometry, and tinnitogram showed normal ®ndings.

Discussion
In our case, right hemiplegia with loss of consciousness developed immediately after partial seizure
episode, but these neurologic de®cits recovered
completely in 15 min after IVMP. As reported,1 ± 5
early vigorous therapy with combined IVMP and
IVCy seemed to be an effective therapy for children
with severe NPSLE.
Interestingly, this patient showed unexplained
redness of ears. `Red ear(s) syndrome' consisted of
many etiologies.6,7 Lance suggested that this syndrome would be associated with irritation of the third
cervical root, temporomandibular joint dysfunction,
thalamic syndrome, or without obvious structural
cause.6 Among these causes, red ears in conjunction
with thalamic syndrome would result from disinhibition of the quintothalamic neuron to produce continuous burning pain in the face, including pain in the
ears.6 Hirsch reported the presence of red ear(s)
syndrome in patients with headache, which may be
caused by an underlying dysregulation of sympathetic
out¯ow.7 O'Gradaigh et al reported swollen tender
indurated red ears and adjacent skin in antiphospholipid syndrome, which resulted from bilateral
thrombosis of ears.9
The etiology of red ears in our patient is obscure.
He did not have any apparent predisposing factor or
etiology as described above.6,7,9 However, even
though it could not be a complete explanation,
thalamic lesion would be the most probable cause of

red ear(s) syndrome in this patient because our patient
showed the vasculitis of left middle cerebral artery
and infarction in left basal ganglia, which might
involve the thalamus.
There has not been any report of red ear(s)
syndrome in SLE or NPSLE patients. Therefore
further observation of the red ear(s) phenomenon in
SLE is needed to clarify the association between them
and the possible pathogenesis.
We suggest that early aggressive therapy with
combined IVMP and IVCy in a child with severe
NPSLE is an effective therapy, and red ear(s)
syndrome would be a part of the unusual manifestation of NPSLE.

303

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Lupus
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