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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 3  |  Page : 155-158

Kawasaki disease - two case reports from a rural set up in Gujarat


Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad, Gujarat, India

Date of Web Publication10-Jul-2015

Correspondence Address:
Pragya A Nair
Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad - 388 325, Gujarat
India
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Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.


DOI: 10.4103/2319-7250.160659

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  Abstract 


Kawasaki disease (KD), also known as the mucocutaneous lymph node syndrome is a diffuse vasculitis of unknown etiology affecting children with a characteristic clinical presentation. It is a disease with a unique clinical presentation. Knowledge about this established disease is required as it can involve the coronary and other medium and small-sized arteries causing vasculitis in about 20% of cases, which can be the cause of ischemic heart disease and sudden deaths in third and fourth decade of life. It is a condition diagnosed entirely on a clinical basis and hence the need of awareness particularly in pediatricians and dermatologists is the need of time, as no of cases are on the rise. We hereby report two cases of KD diagnosed on the basis of history and clinical findings.

Keywords: Kawasaki disease, strawberry tongue, vasculitis


How to cite this article:
Nair PA, Diwan NG. Kawasaki disease - two case reports from a rural set up in Gujarat. Indian J Paediatr Dermatol 2015;16:155-8

How to cite this URL:
Nair PA, Diwan NG. Kawasaki disease - two case reports from a rural set up in Gujarat. Indian J Paediatr Dermatol [serial online] 2015 [cited 2019 Dec 14];16:155-8. Available from: http://www.ijpd.in/text.asp?2015/16/3/155/160659


  Introduction Top


Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and young children. It is characterized by fever, bilateral nonexudative conjunctivitis, erythema of lips and oral mucosa, rash and cervical lymphadenopathy. KD in India (as also in other developing countries) is probably being misdiagnosed as having viral exanthemata (especially measles) and other febrile illnesses.[1]

The diagnosis of this condition is based on recognition of its characteristic clinical features, and exclusion of other diseases, that may have similar manifestations. It is strongly likely to be confused with scarlet fever, measles, other viral exanthemas, drug eruptions and rare disorders like rickettsial/leptospiral fever. Recent data suggest that the incidence of KD is increasing in India.[2][3][4][5][6][7] There is a need for creating more awareness about KD among practicing pediatricians and dermatologist in India particularly in rural set up like us as it is the cause of sudden death in 3rd and 4th decade of life. We hereby presents two cases of KD diagnosed on the basis of clinical criteria.


  Case report Top


We came across two patients in last 1-year period who presented with fever for more than 5 days. Both the patients were diagnosed on the basis of clinical history and lab findings [Table 1] and [Table 2].
Table 1: Clinico-epidemiological profile of patients

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Table 2: Laboratory parameters of patients

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Patients were given tab aspirin, oral antibiotics, and other supplementary drugs. The first patient was followed up for 3 months without any systemic involvement. Patients electrocardiogram (ECG) was normal, and she was kept on oral aspirin, but the second patient was lost in follow up.


  Discussion Top


Kawasaki disease is an acute febrile systemic vasculitis that was first described by Kawasaki et al.[8] in 1974. It is a common pediatric disorder with the annual incidence being in the range of 60–150/100,000 in children below 5 years of age.[9] KD occurs worldwide and affects children of all races, although Asians are believed to be at highest risk.[2],[10] The male-to-female ratio is 1.5:1.0.[11]

Although KD is believed to be caused by an infectious agent in an immunologically susceptible individual, the causative agent remains elusive. Some of the infectious agents proposed are parvovirus, Staphylococcus aureus, Epstein – Barr virus, chlamydia and mycobacteria.[12] The role of staphylococcal and streptococcal superantigens in the etiopathogenesis of KD has been under close scrutiny, especially because some of the clinical features of KD (e.g. exanthem and peripheral desquamation) are reminiscent of a toxic shock syndrome. Onouchi et al.,[13] had suggested that a common infectious agent that triggers the clinically apparent disease in certain genetically predisposed individuals, particularly Asians, causes KD.

The course of KD can be divided into three clinical phases: Acute, subacute and convalescent.[14]



  • Acute febrile phase lasts for 7–14 days, ends with the resolution of fever, is characterized by conjunctival injection, mouth and lip changes, swelling and erythema of the hands and feet, rash and cervical lymphadenopathy


  • Subacute phase the period from the end of the fever to about day 25. Patients have desquamation of the fingers and toes (most characteristic for KD), arthritis and arthralgia, and thrombocytosis


  • The convalescent phase begins when clinical signs disappear and erythrocyte sedimentation rate becomes normal, 6–8 weeks after the onset of illness.




In the absence of a specific diagnostic test, KD is a clinical diagnosis based on the characteristic history and physical findings.[15] The diagnosis of classic KD requires fever of at least 5 days duration and the presence of four of the following:



  • Changes in extremities, e.g., erythema, edema, and desquamation. Desquamation of the fingers and toes begins in the periungual region, may involve the palms and soles, and is usually observed 1–2 weeks after the onset of fever


  • Bilateral conjunctivitis (not associated with exudates)


  • Polymorphous rash (not vesicular)


  • Cervical lymphadenopathy (usually >1.5 cm and unilateral; the least common of all clinical features, occurring in approximately 40%)


  • Changes in the lips and oral cavity (e.g., pharyngeal erythema, dry/fissured or swollen lips, strawberry tongue).




Atypical cases of KD are not uncommon (up to 15–20%). Herein, common supplementary criteria for the diagnosis of incomplete KD are introduced [Table 3]. The AHA criteria (2004),[16] which incorporate suggestions for laboratory tests and early echocardiography, are helpful for diagnosing incomplete KD. Supplementary criteria for diagnosing KD should be applied to every pediatric patient who experiences fever for more than 5 days (with two or three principal clinical features of KD) without a known infection source.
Table 3: Supplementary laboratory criteria for incomplete KD

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Supplementary laboratory criteria for incomplete KD.[17][Figure 1],[Figure 2],[Figure 3]

Fever of >5 days associated with 2 or 3 clinical criteria, C-reactive protein - 3.0 mg/dL and/or erythrocyte sedimentation rate - 40 mm/h with the following criteria:



  • Albumin ≤3.0 g/dL


  • Anemia for age


  • Elevation of alanine aminotransferase


  • Platelets after 7 d ≥450,000/mm3


  • White blood cell count ≥15,000/mm3


  • Urine ≥10 white blood cells/high-power field.




Figure 1: Maculopapular rash over upper part of trunk (case 1)

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Figure 2: Strawberry tongue and exfoliation of lips (a) case 1, (b) case 2

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Figure 3: Exfoliation of skin (case 2) (a) palms, (b) soles

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The differential diagnosis includes toxic shock syndrome (streptococcal and staphylococcal), staphylococcal scalded skin syndrome, scarlet fever, and infection with enterovirus, adenovirus, measles, parvovirus, epstein barrvirus, cytomegalovirus, mycoplasma pneumonia.

Cardiovascular manifestations can be prominent in the acute phase of the illness and are the leading cause of morbidity and mortality in children with untreated KD. Abnormalities may include diffuse ectasia or coronary aneurysms. Cardiac complications other than coronary arterial abnormalities include cardiac tamponade, cardiac failure, myocarditis, and pericarditis, rickettsiae, and leptospirosis.[8]

Echocardiograms and angiograms obtained in long-term follow-up studies indicate that coronary aneurysms resolve within five to 18 months in approximately 50% of patients.

The medical management of KD involves the use of gamma globulin and aspirin as anti-inflammatory agents, oral steroids, statins and long-term anticoagulation. Periodic checkup and counseling for cardiovascular risk factors is recommended for all children who have KD. According to Newburger et al.[17] If 3 supplement criteria are met, intravenous immunoglobulin (IVIG) can be prescribed before performing echocardiography.

Both of our patients were labeled as classical KD as both presented with fever for more than 5 days with maculopapular rash, swollen lips and strawberry tongue, lymphadenopathy, which was generalized in case-1 and only cervical in case-2 with exfoliation of skin from palms and soles. Total count, erythrocyte sedimentation rate and C-reactive were high in both the cases. As patients were not ready for echocadiography, they were made aware about the chances of cardiovascular involvement and importance of quarterly ECG checkup. Patients were managed with oral aspirin, antibiotics, and other supportive drugs. IVIG was not started due to nonaffordability on the part of both of them.

In such a scenario, improving awareness amongst practicing pediatricians and dermatologists across the community might facilitate early diagnosis and thereby possibly prevent coronary sequelae.

 
  References Top

1.
Balasubramanian S, Krishna MR, Dhanalakshmi K, Amperayani S, Ramanan AV. Factors associated with delay in diagnosis of Kawasaki disease in India. Indian Pediatr 2012;49:663-5.  Back to cited text no. 1
    
2.
Paul DK, Gupta A, Lahiri M. Kawasaki disease in Calcutta. Indian Pediatr 2000;37:1264-5.  Back to cited text no. 2
    
3.
Singh S, Kansra S. Kawasaki disease. Natl Med J India 2005;18:20-4.  Back to cited text no. 3
    
4.
Singh S, Gupta A. Kawasaki disease – A common cause of acquired heart disease in India. Indian Heart J 2004;56:261-2.  Back to cited text no. 4
    
5.
Singh S, Bansal A, Gupta A, Kumar RM, Mittal BR. Kawasaki disease: A decade of experience from North India. Int Heart J 2005;46:679-89.  Back to cited text no. 5
    
6.
Singh S, Gupta MK, Bansal A, Kumar RM, Mittal BR. A comparison of the clinical profile of Kawasaki disease in children from Northern India above and below 5 years of age. Clin Exp Rheumatol 2007;25:654-7.  Back to cited text no. 6
    
7.
Singh S, Aulakh R, Bhalla AK, Suri D, Manojkumar R, Narula N, et al. Is Kawasaki disease incidence rising in Chandigarh, North India? Arch Dis Child 2011;96:137-40.  Back to cited text no. 7
    
8.
Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974;54:271-6.  Back to cited text no. 8
    
9.
Sundel RP, Petty RE. Kawasaki disease. In: Cassidy JT, Petty RE, Laxer RM, Lindsley CB, editors. Textbook of Pediatric Rheumatology. 5th ed. Philadelphia: Elsevier Saunders; 2005. p. 521-38.  Back to cited text no. 9
    
10.
Mitra S, Singh S, Grover A, Kumar L. A child with prolonged pyrexia and peripheral desquamation: Is it Kawasaki disease? Indian Pediatr 2000;37:786-9.  Back to cited text no. 10
    
11.
Singh S, Kawasaki T. Kawasaki disease – An Indian perspective. Indian Pediatr 2009;46:563-71.  Back to cited text no. 11
    
12.
Yeung RS. Kawasaki disease: Update on pathogenesis. Curr Opin Rheumatol 2010;22:551-60.  Back to cited text no. 12
    
13.
Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M, et al. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. Nat Genet 2008;40:35-42.  Back to cited text no. 13
    
14.
Bhardwaj P, Sharma VK. Fever and rash: It can be Kawasaki disease. Indian J Dermatol 2009;54:29-31.  Back to cited text no. 14
  Medknow Journal  
15.
American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. Diagnostic guidelines for Kawasaki disease. Am J Dis Child 1990;144:1218-910.  Back to cited text no. 15
    
16.
Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. 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 2004;110:2747-71.  Back to cited text no. 16
    
17.
Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki. Dis Arch Pediatr Infect Dis 2013;1:159.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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