EDITORIAL COMMENTARY

Kawasaki Disease: Unusual Clinical Manifestations

Lesa Dawman & S. K. Kabra

Received: 20 February 2014 /Accepted: 3 March 2014 /Published online: 16 March 2014# Dr. K C Chaudhuri Foundation 2014

Kawasaki disease (KD) is an acute febrile systemic vasculitisof unknown etiology and was first described in 1967 in Japan[1] and later in the English-language literature in 1974 byTomisaku Kawasaki, who suggested the name mucocutane-ous lymph node syndrome [2].

No definitive diagnostic laboratory test exists for KD [3, 4].Diagnosis of KD is based on constellation of clinical featuresand supportive laboratory findings.

In this issue of IJP; Singh et al. discuss about missing KDduring childhood and subsequent coronary artery disease(CAD) in adulthood [5]. They proposed that if incidence ofKD is 4.54/100,000 in children below 15 y of age, about17417 cases of KD may occur annually in India. In absenceof authentic data it is difficult to confirm this figure but it canbe assumed that majority of these patients are missed [6]. Thisraises an important hypothesis that identifying KD duringchildhood and treating them with appropriate interventionmay reduce risk of CAD in adults. It is important to under-stand that epidemiology of KD depends on several factorsincluding genetic susceptibility, ethnic background and envi-ronmental triggers. The incidence varies widely across coun-tries and within different ethnic groups [7]. Therefore, it isimportant to confirm this hypothesis by carefully designedstudies in India to formulate policy for early diagnosis andintervention.

In this issue of Indian Journal of Pediatrics (IJP) multipleunusual/atypical manifestations with KD are reported. Zhanget al. report a child with KD with a localized neuroblastoma(NB) [8]. The child presented with typical clinical fea-tures of KD in the form of fever, puffy red eyes, red

fissured lips, strawberry tongue, mild swelling andperiungual desquamation. ECHO showed dilatation ofleft and right coronary artery. After receiving IVIgG,his fever improved but recurred 5 d later; the ultrasoundstudy revealed an abdominal mass in her left adrenalgland, that was diagnosed as poorly undifferentiatedneuroblastoma with fine needle biopsy. Association ofneuroblastoma and KD has been reported earlier [9]. Toestablish KD as a new paraneoplastic syndrome withneural crest tumors, needs more experience and reportof follow up of these patients along with immunologicalstudies.

Gupta et al. described their experience of managing twochildren with giant aneurysms (GA), a rare manifestation ofKD [10]. First patient, a 3-y-old girl had typical clinicalfeatures of KD like, high grade fever, generalizedmaculopapular rash and redness of eyes, lips and tongue.She improved with intravenous immunoglobulin (IVIgG)and high dose aspirin. Initial response was followed byrecurrence of symptoms that responded to repeat dose ofIVIgG and infliximab. Another child, a 2-y-old boy pre-sented with high grade fever of 9 d duration with non-purulent conjunctivitis, erythematous oral mucosa and dis-crete non-tender left cervical lymphadenopathy. He alsorequired two doses of IVIgG and infliximab for improve-ment. ECHO cardiography showed giant aneurysms in bothof the cases. Kawasaki disease and giant coronary aneu-rysm has been reported earlier; available information sug-gests that these patients require long term treatment andfollow up [11, 12].

Madhusudan et al. report a 5-y-old child with typicalclinical features of KD who received IVIgG with improve-ment in the beginning [13]. Subsequently, he developed fever,pedal edema cardiomegaly and dilated left ventricle withejection fraction 30–35 % with mild mitral regurgitation.Child improved with systemic steroids. Myocarditis during

L. Dawman : S. K. Kabra (*)Department of Pediatrics, All India Institute of Medical Sciences,New Delhi 110029, Indiae-mail: skkabra@live.in

Indian J Pediatr (April 2014) 81(4):325–327DOI 10.1007/s12098-014-1397-4

acute stage has been described in KD [14], however long termabnormalities are less frequent. In this report authors do notmention investigations including work up for viral infectionsto rule out incidental viral infection causing myocarditis. Amyocardial biopsy might add to better understanding of thiscomplication. Therefore, this is important to document latemyocarditis in KD but it remains an association rather than acause of KD.

Khubchandani et al. describe a girl with KD and isolatedfacial nerve palsy on day 27 that resolved after 3 wk offollow up [15]. Diagnosis of KD was based on constellationof features including fever, irritability, conjunctival injec-tion, strawberry tongue, cracked lips, edematous handsand feet and perianal excoriation and echocardiographyshowing diffuse dilatation of all the coronary arteries andaneurysm of left anterior descending and proximal rightcoronary artery with minimal pericardial fluid. She receivedIVIgG twice and her symptoms resolved completely. Facialnerve palsy has been reported rarely in children with KD[16]. Though authors document clinical recovery withIVIgG they do not mention details of investigations includ-ing electrophysiologic studies to relate nerve injury/ischemia and its recovery with treatment. There is possibil-ity of vasculitis causing nerve injury due to ischemia,however, in absence of objective evidence it may be con-sidered as association rather than cause of facial palsy.

There are multiple reports of association of KD with otherclinical features including pancreatitis at the onset of KD [17],KD mimicking retropharyngeal abscess [18], acute appendi-citis as the presenting manifestation of KD [19], unresolvingpneumonia as the main manifestation of atypical KD [20],acute exudative tonsillitis and bilateral cervical lymphadenitis[21], erythema multiforme as the first sign of incomplete KD[22] and kidney and urinary tract involvement in KD [23],reaction at the site of tuberculin skin test given a month ago[24], anterior uveitis [25], submandibular abscess as present-ing manifestation of KD [26] and Henoch–Schönlein purpurawith KD [27]. In most of the reports, authors discuss associ-ation by circumstantial evidence rather than trying to establishcausal relation with detailed work up.

With increasing awareness, diagnosis of KD is made moreoften and multiples cases with associated rare manifestationshave been reported. In the absence of diagnostic tests andspecific clinical features it is difficult to make diagnosis ofKD and to establish causal association of various atypical/unusual clinical features with KD. In the absence of biologicalexplanation for association of these atypical features they mayremain incidental occurrence. Therefore, it is crucial to inter-pret such reports carefully.

Conflict of Interest None.

Role of Funding Source None.

References

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