A 4-month-old girl with fever and rash

Prepared by....... Rekwan Sittiwangkul, M.D
CC : High fever for 4 days.
PI : Four days prior to admission (PTA), she developed high fever and rhinorrhea. She was taken to see a doctor at a clinic and was diagnosed as "URI". She was prescribed paracetamol, actifed and amoxicillin . Two days PTA, she had semi-solid stool. One day PTA, she developed maculopapular rash at trunk at extremities.
PH : she had been healthy . Birth weight was 2.8 kgs.
Significant PE

An infant, fully conscious but irritable, body weigh 5.2 kgs.
Vital signs: Temp 39.8 C, HR 160 beats/min, RR 40 breaths/min, BP 98/50 mmHg
HEENT: mild injected conjunctiva, no icteric sclera, red lips (Figure 1), normal anterior fontanel.
Lymph node: not palpable
Heart: regular rhythm, tachycardia
Lung: clear, no adventitious sound
Abdomen: soft, no organomegaly
Skin : Erythematous rash at trunk and extremities , Indurated and redness of old BCG scar at left shoulder (Figure 1)
Extremities: Swelling of dorsal part of hands and feet (Figure 2,3)

Figure 1: Red lip, erythematous maculo-papular rash on face and upper extremities, indurated and redness of BCG scar. Figure 2: dorsal swelling of hands
Figure 3: dorsal swelling of feet
Investigations:
CBC: Hb 10.6 gm%, Hct 30.7%, WBC 14,000/cumm (N 72%,L 28%), platelets count 328,000/cumm
PBS: normochromic normocytic RBC, toxic granulation 1+, vacuolization 1+
U/A: yellow ,clear, spgr 1.024, PH=5, WBC 20-30/HPF, albumin 1+
ESR: 97 mm. at 60 minutes (normal 0-20 mm.)
EKG: sinus tachycardia , rate 170/min, no ST-T change
Echocardiogram: No structural heart defect, normal left ventricular function (EF 67%), minimal pericardial effusion 5mm. At apex without any fibrin. Normal size coronary arteries (proxmal left coronary artery= 1.8 mm, right coronary artery 1.7mm)
Course in the hospital : ( Figure 4)

On the first admission day, while waiting for U/C, she was started on intravenous gentamicin for a working diagnosis of "UTI". Repeated single catheter for urine exam for gram stain was done. The 2nd U/A revealed many WBC in urine and urine gram stain was negative. On 2nd day of admission, after getting all investigations and the persistent clinical symptoms, the diagnosis was changed to Kawasaki disease. Then she was given a high dose intravenous immunoglobulin (IVIG) 2g/kg and high dose and started on oral aspirin ( 80mg/kg/day). Four hours after IVIG the fever dramatically subsided as in figure 4.

Two days after IVIG ,her irritability, erythematous rash, red lip and edema of extremities dissappeared. She was discharged home with low dose aspirin (5mg/kg /day).

Other investigations:
U/C: (3 days later) : no growth
H/C: (5 days later) : no growth

Follow up course( 2 weeks later) :
She had peeling of skin at finger base as shown in figure 5. Investigation showed her platelets count was 955,000/cumm and her ESR at 60 minutes came down to 27mm. Her repeated echocardiogram showed mild dilatation of proximal left coronary artery (3.5mm) without any thrombus. she was put on continued low dose aspirin and her next follow up was 6 week later .
Final diagnosis : Kawasaki disease (responsive to IVIG treatment) with mild dilatation of left coronary arteries.
Note:
1. Kawasaki should be included in differential diagnosis of all cases who presented with fever with rash.
2. Other main criteria (red lip, edema of extremities, injected conjunctiva )are helpful for diagnosed Kawasaki in this case. Cervical lymphadenopathy was less helpful (incidence only 50%).
3. Other associated finding such as extremely irritable, indurated and erythema of BCG scar ,and sterile pyuria from urine exam should alert physician to suspected Kawasaki disease.
4. Every patients diagnosed Kawasaki disease should be undergo echocardiogram evaluation in 2-3 weeks after onset of disease due to high incidence of coronary artery abnormalities( 25 % without IVIG treatment ,5 % in IVIG treatment) which may leading to sudden death

 

Diagnosis : Kawasaki disease

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