Kawasaki syndrome 
Case report 
MUCOCUTANEOUS LYMPH NODE SYNDROME 
(KA W ASAKI SYNDROME) 
l. Orojan and L. Tarok 
ABSTRACT 
The authors present a Kawasaki syndrome in an adolescent, which is very rare. He failed to respond to 
antibiotic treatment with amoxicillin, but did respond to salicylates and antihistaminics. Thanks to the early 
diagnosis and the effective treatment, later complications could be avoided. 
KEY WORDS 
mucocutaneous lymph node syndrome, Kawasaki, early diagnosis, treatment with salicylates 
INTRODUCTION 
The mucocutaneous lymph node syndrome was 
first reported by Kawasaki in 1967. This disease 
occurs primarily in childhood. The typical clinical 
symptoms are high fever of the continua-type, indu-
rative edema of the extremities, palmar and plantar 
erythema, polymorphous generalised exanthema, enan-
thema of the labial and oro-pharyngeal mucosa, 
bilateral conjunctivitis, acute non-purulent unilateral 
cervical lymphadenopathy and desquamation of the 
skin on the finger tips during the regression of the 
disease (1,2). 
We present the classical Kawasaki syndrome in an 
adolescent, which is quite rare at this age. 
CASE REPORT 
A 16-year-old male patient was registered at our 
department 4 days after the appearance of the first 
acta dermatovenerologica A.P.A. Vol 6, 97, No 1 
clinical symptoms. He complained of an infection of 
the upper respiratory tract accompanied by high 
fever (38-39°C), lesions of the mucous membranes 
and an unilateral lymph node hypertrophy on the 
neck. He failed to respond to the treatment 
(amoxicillin, paracetamol, diphenhydramin) reco-
mmended by his family doctor and by the specialist 
for interna! diseases. Additionally, two days later a 
disseminated exanthema and edema of the extremities 
appeared. The family-anamnesis was negative, his 
parents mentioned only a pneumonia in early child-
hood, till the present disease he enjoyed a good 
health. 
State at admission: numerous, disseminated, well 
defined maculopapules 0.2-2 cm in size, which extended 
to palms and soles. On the lower extremities there 
were point-like petechias. The lips were inflammed, 
red and swollen; there were some rhagades.The oral 
mucosa was inflammed too, the conjunctivae were 
injected (Fig.1-3). 
31 
Kawasaki syndrome 
Fig. l. Bilateral conjunctivitis 
Other findings: covered right lung on the X-ray pictures, hepatomegalia, 
tachycardia and aching and restricted movements of extremities. In the 
right occipital region a painful hypertrophic and mobile lymph node 
was palpable. 
Routine laboratory tests: ESR 20 mm/h, slight anaemia:hemoglobin 7.72 
mmol/1, hematocrit 0.358, slight leukocytosis 98x 109/1, increased values 
of Jiver enzymes: SGOT 75 U, SGPT 54 U, decreased value of 
thrombocytes 89xl09/l, and an increased value of fibrinogen 4.9lxl09/l. 
The differential blood count was (% ): non-seg O, seg 74, basophils O, 
eosinophils O, monocytes O, lymphocytes 26. Tota] protein: 79 g/1. 
Protein electrophoresis: albumin 0.636, a 1 0.036, a 2 0.073, ~ 0.091, y 
0.162. 
Fig. 2. Edematous, inflammed lower lip with fissures 
Tests for viruses: HIV, parvo, adeno, 
morbilli, rubella, Epstein-Barr virus 
were negative. The Hbs antigens, 
hepatitis C virus antibody, mono-
sticon tests and immunoelectro-
phoresis were also negative. Patho-
genic agents from the larynx mucosa 
could not be cultivated. The abdo-
minal ultrasound examination indi-
cated hepatosplenomegalia and diffu-
se liver damage. The ECG showed 
no cardial complications and the 
result of the echocardiography exami-
nation was also negative. 
Course oj the disease and treatment: 
high fever (38-39°C) was registered 
during 3 days before and 3 days 
after the admission to our depart-
ment. We induced the therapy with 
salicylates, antihistamines ( clemastin, 
loratadin) and indometacin. As a 
result of the therapy, patient's 
continuous high temperature gradu-
ally receeded and his articular pain 
diminished significantly. Due to the 
externally applied antiphlogistic 
soothing treatment, the inflammatory 
symptoms of the skin and mucosa 
regressed. Our diagnosis was based 
on the existing symptoms at the 
admission. The diagnosis was confir-
med also by the course of the 
disease. Further typical symptoms 
were the desquamation of the skin 
on the finger tips and the regression 
of the unilateral adenopathy on the 
neck in the second week of the 
disease (Fig 4). 
Because of the early diagnosis 
and the immediate treatment with 
salicylates no cardial complications 
were found either by echocardi-
ography during the stay in the clinic 
· nor 8 weeks later. 
DISCUSSION 
Kawasaki was the first to describe 
the above-mentioned disorder as 
mucocutaneous lymph node syn-
drome. Today it is referred in the 
literature as the Kawasaki syndrome. 
32 acta dermatovenerologica A.P.A. Vol 6, 97, No 1 
The number of reports the disease 
occurring at different ages bas been 
increasing. It bas been reported 
primarily in childhood, more often 
in boys than in girls (2:1). In some 
of these cases the disease was 
identified only in the late com-
plicated stages (3,4,5). As far as 
we know in Hungary only pediatric 
cases and only one in an adult 
patient was published (6) . Reports 
which describe an atypical course 
are also increasing (7). 
The etiology of the disease is 
not quite clarified. On the basis of 
the frequent epidemic-like occurrence 
a biological agent as a cause bas 
to be assumed (8). 
The provocative roles of the viruses 
as Epstein-Barr, retro- and hepatitis 
B as well as Y ersiniae, Rickettsiae, 
acarus and even of heavy-metal 
toxaemia were discussed but were 
not praven yet. The !atest research 
tries to find the role of the parvo-
virus B 19 infection (l,2,9,10,11,12). 
Earlier it was believed to be a 
variant of polyarteritis nodosa juve-
nilis. Besides the immunological fac-
tors the cytokins seem to play a 
significant role in the pathomecha-
nism of the disease. Thus the TNF-
alpha can be blamed tor the vascular 
changes. The production of the 
interleukin-2 and interleukin-6, the 
T-cell activity and the megakaryocyte 
proliferation seem to be on an 
increase. The interferon-gamma helps 
to present the new antigen to the 
macrophages. 
During the disease the blood leve! 
of the immunoglobulins, the number 
and concentration of antiendothelial 
and antineutrophil antibodies incre-
ase. It is important to mention 
that the frequency of coronary com-
plications shows correlation to con-
centration of cytokines in the tissues. 
(13). The recently drawn criteria 
help to diagnose the disease (14,15). 
Nowadays the superantigens are 
raised as the causal factor. 
Kawasaki syndrome 
Fig. 3. Maculo-papular exanthema on the trunk 
Fram differential diagnostic point of view, scarlatina, measles, rubella, 
erythema multiforme, hepatitis, juvenile rheumatoid arthritis, Steven-
Johnson syndrome, infantile polyarteritis nodosa and toxic shock should 
be taken into consideration. 
The serious complication of the syndrome are the coronary thrombosis 
and aneurysm. Death sets in due to aneurysm rupture or to infarction. 
Deviations, which can be registered by ECG can occur in the first or 
in the second week of the acute phase. Vasculitis can develop, the 
media of the vessels is destroyed and an inflammatory infiltration in 
the intima and in the surrounding connective tissue can be observed. 
On rare occasions cases of aseptical meningitis (5) and an obstruction 
of the arteria cerebri media were noted (16). 
Fig. 4. Typical desquamation of the skin, localized on the finger tips 
acta dermatovenerologica A.P.A. Vol 6, 97, No 1 33 
Kawasaki syndrome 
CONCLUSION 
To avoid complications and to obtain a successful 
therapy, it is necessary to make a quick diagnosis 
and to induce an early treatment with salicylates, 
gamma-globulin or perhaps steroids. In our case, 
neither the ECG nor the echocardiography during 
the early and late stages did prove coronary damage. 
However, though our patient was already 16-year-
old and thrombocytosis was not found (17), we 
could make the diagnosis on the basis of the 
following symptoms: high fever of the continua-type 
for more than 5 days, bilateral conjunctivitis, injected 
lips and pharynx, fissured lips, edematous erythema 
of hands and legs, a polymorphous and disseminated 
exanthema as well as cervical lymphadenopathy. 
The desquamation of the skin in phalangeal regions 
of the hands started in the 2nd week of the 
disease. 
We could make the diagnosis of the Kawasaki 
syndrome in spite of the age (6) of the patient and 
of the moderate thrombocytopaenia. 
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AUTHORS' ADDRESSES 
34 
Ivan Orojan MD, Department of Dermatology, County Hospital Bacs-Kiskun, H-6000 Kecskernet, 
Nagyk6r6si u. 15. Hungary 
med. babi!. Laszl6 Tarok MD, Head physician, same address 
acta dennatovenerologica A.P.A. Vol 6, 97, No 1