Forum for Nordic Dermato-Venereology Nr1,2019 | Page 28

Luit Penninga, Anne Kathrine Lorentzen and Carsten Sauer Mikkelsen – Kawasaki Disease: Two Episodes of Recurrent Disease in a
Greenlandic Inuit Boy
90% of cases (4). Oral mucous membrane symptoms in 90%,
symptoms in the extremities in 50–85%, ocular symptoms in
more than 75%, and cervical lymphadenopathy in 25–70% (1).
This means that not all symptoms and clinical manifestations
occur in all patients with Kawasaki disease; symptoms do not
always come in the same order, and are not always present at
the same time. Due to the very high occurrence of cutaneous
rash and oral mucous symptoms, dermatologists are frequently
consulted (3, 4).
The rash often appears in the early phase of the disease,
typically as erythema in the region of the perineum, and
desquamation. This is followed by morbilliform, targetoid or
macular skin lesions on the torso and extremities (3). Kawasaki
disease may initiate a psoriasiform eruption in children not
previously diagnosed with psoriasis. Vesicles and bullae are
normally not seen with Kawasaki disease. Patients may have
redness and crust formation at the site of the Bacille Calmette
Guerin (BCG) vaccination (3, 4). This is, of course, only rele-
vant in countries, such as Greenland, where BCG vaccination
is part of the childhood immunization programme.
Fig. 3. Erythema of the lips and oral mucosa, slight rash, and bilateral
non-exudative conjunctivitis.
in the extremities, bilateral non-exudative conjunctivitis and
cervical lymphadenopathy. The same day he was treated with
IV immunoglobulins, and shortly afterwards, all symptoms had
disappeared. As recurrence of Kawasaki disease is rare, he was
tested for immunological defects and other genetic diseases,
but none were found. After 5 months, a new episode with
fever and the classic features of Kawasaki disease occurred.
Once again treatment with IV immunoglobulins was given,
and he recovered quickly. As this was the second episode of
recurrent Kawasaki disease, he again underwent investigation
for immunological diseases, but all tests again were negative. A
repeat echocardiography showed normal myocardial function,
and no progression of the coronary artery dilatation. At fol-
low-up one year after the initial episodes of Kawasaki disease,
no further episodes had occurred.
D iscussion
Fever is common during childhood due to infectious diseases.
However, fever due to systemic inflammation is also one of
the main features of Kawasaki disease (1). Kawasaki disease
should be considered in children who have unexplained fever
for more than 5 days (8).
Kawasaki disease can be diagnosed by the presence of typical
clinical manifestations. A polymorphous rash is seen in 70–
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C ase R eport
Changes on the extremities occur in the final phase of the dis-
ease. Symptoms include indurated oedema on the dorsal side
of the hands and feet, and diffuse erythema of the palms and
soles. Recovery from Kawasaki disease is associated in 68–98%
of children with sheet-like desquamation of the periungual
hand and feet regions. Furthermore, linear nail creases occur,
also called Beau’s lines (1).
Oral mucous membrane symptoms include red, cracked lips
and a strawberry tongue. The strawberry tongue is caused by
sloughing of filiform papillae and denuding of the inflamed
glossal tissue. Often these symptoms become more evident
as Kawasaki disease progresses. Discrete oral lesions, such as
ulcers, vesicles, or tonsillar exudate, are suggestive of condi-
tions other than Kawasaki disease (1).
Arthritis is not part of the diagnostic criteria of Kawasaki
disease, but occurs with either oligoarticular or polyarticular
involvement in 7–25% of cases (1).
Cardiovascular symptoms and complications of Kawasaki
disease, although not part of the diagnostic criteria, may cause
severe morbidity and mortality. At the time of diagnosis, 30%
of patients have dilatation of the coronary arteries.
It is important to highlight that there is no single laboratory
test that can confirm or deny the diagnosis of Kawasaki disease.
Kawasaki disease causes systemic inflammation, and increases
in C-reactive protein and erythrocyte sedimentation rates are
frequently seen, as well leukocytosis and thrombocytosis (2).
Forum for Nord Derm Ven 2019, Vol. 24, No. 1