Rheumatology Advance Access originally published online on July 15, 2006
Rheumatology 2006 45(11):1444-1445; doi:10.1093/rheumatology/kel194
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Severe cutaneous manifestations in a child with refractory Kawasaki disease
Department of Pediatrics, Rheumatology Unit, University of Firenze and 1Cardiology Unit, A. Meyer Children's Hospital, Firenze, Italy
Correspondence to: Fernanda Falcini, MD, Department of Paediatrics-Rheumatology Unit, Via Pico della Mirandola 24, 50132 Firenze, Italy. E-mail: falcini{at}unifi.it
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Introduction |
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SIR, We report a child with refractory Kawasaki disease (KD), who presented severe scattered crusting skin lesions as predominant manifestation of the disease.
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Case report |
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A 17-month-old boy presented to our department with a 3 day history of fever reaching 39°C, resistant to amoxicillin, along with a maculopapular rash on the face, trunk and limbs. Past medical history was unremarkable. Routine laboratory work-up revealed erythrocyte sedimentation rate (ESR) 72 mm/h, C-reactive protein (CRP) 13.67 mg/dl (nv < 0.35), haemoglobin 10 g/dl, white blood cell count 26.50 x 103/mm3, fibrinogen 837 mg/dl and sodium 129 mEq/l (Table 1). Microbiological evaluation for bacterial and viral infections, including adenovirus, cytomegalovirus, parvovirus, herpes and Epstein–Barr virus, Staphylococcus and Group A Streptococcus, were negative. Throat, nasopharyngeal and cutaneous swabs for culture were also negative. Chest X-ray and abdominal ultrasound were unremarkable. On the day after admission, he developed non-exudative conjunctivitis, cervical lymphoadenopathy and mucositis, and KD was suspected. Echocardiogram revealed normal systolic and diastolic left ventricular dimensions (29/19 mm), with normal fractional shortnening (32%) and ejection fraction (56%). The diameter of the left coronary artery was increased: 3.6 mm (z-score size for age: 2.5 mm). Intravenous immunoglobulin (IVIG) and aspirin were promptly administered on day 5 from the fever onset. Notwithstanding, fever rose up to 40°C and a significant worsening of the skin lesion occurred: itching and burning tender rash all over the body, scalp included. Cracked and scabbed lips appeared. A further IVIG cycle resulted unsuccessful in subsiding fever and cutaneous manifestations that later also presented blisters at the ankles and ear lobes. The ESR and CRP were still raised and platelet count rose up to 771 x 103/mm3. On day 8, an echocardiogram confirmed the previously reported coronary lesion. Despite a third dose of IVIG on day 10, fever persisted and diffuse scabs progressively involving cheeks, forehead, eyelids and legs occurred (Fig. 1). Intravenous methylprednisolone (MP), 30 mg/kg, was then given on day 11, but two additional steroid pulses were required over the following days, due to the persistent spiking fever along with elevated inflammatory parameters. On day 16, fever dropped and skin alterations significantly improved. Peeling at fingers and toes was then noted. No changes were detected on echocardiogram and the boy was discharged on aspirin (3 mg/kg). At 1 month follow-up, he had complete resolution of skin lesions and echocardiogram showed normal coronary artery diameter. Artery peripheral involvement was excluded by systemic echo Doppler evaluation.
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Discussion |
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Our patient's case is interesting since the skin involvement predominated the other manifestations of KD through the course of the disease. In addition, he was resistant to current therapeutic regimen.
An erythematosus rash, usually appearing during the acute phase, is part of KD diagnostic criteria. Commonly transient, possibly missed, it may also last 1 week or more. It is reported as non-pruritic, non-vesicular, non-bullous and non-crusting, but possibly purpuric [1, 2]. However, the occurrence of a pustulo-vesicular skin eruption in KD has been recently reported in a 4-yr-old boy [3].
In our case, the rash was typically maculopapular at admission, but over time, it became burning, itching and then vesicular-like and crusting. This eruption suggested Steven–Johnson syndrome as a possible diagnosis, but, in our case, the clear sparing of oral and anal orifices was in contrast. Otherwise, laboratory evidences for considering an infectious disease were also lacking. Persistent spiking fever, conjunctivitis, cervical lymphoadenopathy and mucositis along with coronary artery dilatation prompted us to introduce appropriate therapy for KD. However, fever and skin lesions subsided just after three IVIG and three MP pulses, sequentially administered.
To reduce the rate of coronary involvement, current clinical guidelines claim prompt IVIG administration in case definition suggestive for KD, even if the criteria are not fulfilled, mostly in the presence of coronary lesions [1, 2]. Despite timely introduction of IVIG, 10–15% of the patients do not respond [4–7], and, due to the high risk of coronary sequelae related to long-lasting fever, alternative therapy are considered [1, 2].
Guidelines for refractory patients are still lacking and different regimens have been proposed. An additional 2 g/kg of IVIG therapy is successful in all the patients fail to respond to the first infusion, but 4% of them fails to defervesce as well, and either a third IVIG cycle or corticosteroids are recommended [4–9]. In our patient, a third IVIG cycle was unsuccessful and three successive MP pulses were required to obtain a sustained resolution of fever along with skin recovery.
Timing and use of steroid therapy is still under debate in KD, but it should be considered if there is no response to two or three previous standard doses of IVIG [7–9].
Several anecdotal case series as well as small cohort report successful results of cyclophosphamide, ciclosporin, ulinastatin and infliximab for KD patients resistant to IVIG and corticosteroid regimes [1, 2, 10]. The experience is still anecdotal and no firm conclusions may be drawn before controlled trials are available.
In our patient, since the signs and symptoms of the disease subsided with the last MP pulse, and coronary artery lesions completely disappeared, the decision for an immunosuppressant/biologic drug use was referred and further considered no more.
The authors have declared no conflicts of interest.
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References |
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- Newburger JW, Takahashi M, Gerber MA, et al. (2004) Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 110:2747–71.
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