|Year : 2013 | Volume
| Issue : 1 | Page : 26-29
An infantile Kawasaki disease presenting with pseudo obstruction
KM Sudhakar Rao, M Varna Naidu, Balachandra Ankad, VV Sampgavi
Department of Dermatology Venereology and Leprology, S. Nijalingappa Medical College and HSK Research Centre, Bagalkot, Karnataka, India
|Date of Web Publication||23-Aug-2013|
K M Sudhakar Rao
Department of Dermatology Venereology and Leprology, S. Nijalingappa Medical College and HSK Research Centre, Bagalkot, Karnataka
Source of Support: None, Conflict of Interest: None
Kawasaki disease is a multisystem vasculitis predominantly involving skin and cardiovascular system. In our patient, a 35-day-old female infant presented with fever, abdominal distension, altered sensorium, seizures of 6 days duration. The baby was not responding to conventional antibiotics. Subsequently, she developed rash with vesicles over the perineum and over the palms and soles, edema of hands and feet and fissured lips. The rash appeared 4 days after the onset of systemic symptoms. As per the American Heart Association guidelines for Kawasaki disease, our patient had a fever of 6 days duration and four of the five diagnostic criteria for Kawasaki disease and hence it was a case typical of Kawasaki disease. On investigation, cerebrospinal fluid showed features of aseptic meningitis, urine examination suggested sterile pyuria, with no electrolyte imbalance and no demonstrable foci of infection. Hence, we hereby are presenting a very rare case of Kawasaki disease in a female infant of 35 days; probably the youngest reported so far from India.
Keywords: Aseptic meningitis, Kawasaki disease, pseudo obstruction
|How to cite this article:|
Sudhakar Rao K M, Naidu M V, Ankad B, Sampgavi V V. An infantile Kawasaki disease presenting with pseudo obstruction. Indian J Paediatr Dermatol 2013;14:26-9
|How to cite this URL:|
Sudhakar Rao K M, Naidu M V, Ankad B, Sampgavi V V. An infantile Kawasaki disease presenting with pseudo obstruction. Indian J Paediatr Dermatol [serial online] 2013 [cited 2020 Oct 25];14:26-9. Available from: https://www.ijpd.in/text.asp?2013/14/1/26/116853
| Introduction|| |
Kawasaki disease originally was called as mucocutaneous lymphnode syndrome. It was recognized in Japan by Dr. Tomisaku Kawasaki in 1967.  It is an acute multisystem vasculitis that particularly affects infants and children. Incidence of disease presenting with gastrointestinal manifestations is 2.3%. 
Kawasaki disease is a triphasic illness with an acute phase of 7-14 days, sub-acute phase approximately of 25 days and convalescent phase of nearly 70 days. Nearly 80% of the patients are younger than 5 years, commonly between 18 and 24 months. The male to female ratio is 1.5:1. It is most prevalent in Japan. 
According to the American Heart Association guidelines (AHA), the diagnosis of Kawasaki disease is made when the patient has fever lasting for 5 or more days without other reasonable explanation and fulfills at least four of the five following criteria:
Associated clinical features of Kawasaki disease include:
- Cardiovascular-myocarditis, arterial aneurysms, pericarditis, aortic, mitral regurgitation, ventricular arrhythmias
- Arthralgia and arthritis 
- Urethritis with sterile pyuria
- Aseptic meningitis
- Hydrops of the gall bladder
- Diarrhea, vomiting or abdominal pain
- Sensorineural hearing loss
- Hepatic dysfunction.
Kawasaki disease is based on clinical features that overlap with other illness, diagnostic dilemma can arise. If fever of more than 5 days duration with less than 4 of the 5 criteria as per AHA guidelines and demonstrable coronary artery abnormalities, then it is considered atypical syndrome [Table 1]. 
| Case Report|| |
A 35-day-old female infant presented in the pediatric department with the complaints of fever of 6 days duration associated with altered sensorium, seizures, distension of the abdomen and constipation. Subsequently, the mother noticed erythematous rash and vesicles over the perineum on the 4 th day, erythema over the palms and soles and edema of the hands and feet. There was no history of any prior medication, diarrhea or vomiting.
On examination, the baby was febrile with tachycardia, irritable and poorly feeding. Cervical lymphadenopathy, single lymphnode, measuring about 2 cm in diameter was noted. Cutaneous examination showed fissured lips [Figure 1], erythema of palms and soles, edema of hands and feet [Figure 2] and [Figure 3], erythema, edema and vesicles on the perineum [Figure 4]. There was also polymorphic rash over the distal part of both upper and lower limbs.
|Figure 2: Edema of the dorsum of hand with polymorphic rash over the forearm. Distension of the abdomen is also seen|
Click here to view
|Figure 3: Erythema and edema of feet, perineal erythema, edema and vesicles along with polymorphic rash of both lower extremities|
Click here to view
|Figure 4: Erythema, edema and vesicles over the perineum extending to the thighs with polymorphic rash over the lower extremities|
Click here to view
Desquamation of the skin over the perineum was noted after 2 days.
On Per abdomen examination of the infant, there was an umbilical granuloma and distension of the abdomen [Figure 2] with decreased bowel sounds. On investigation, complete blood analysis showed leucocytosis, raised erythrocyte sedimentation rate (ESR), urine examination showed 6-8 pus cells/high power field with culture showing no growth indicative of sterile pyuria. On cerebrospinal fluid (CSF) analysis, there was a decrease in the sugar level and elevated protein levels. CSF culture was negative for both fungi and bacteria indicating aseptic meningitis. Liver and renal function test were within the normal limits. Ultrasound of the abdomen showed mild ascites. X-ray abdomen showed dilated bowel loops suggestive of paralytic ileus [Figure 5].
Electrocardiogram could not be carried out since infant was irritable. No coronary abnormalities were detected in Echocardiogram.
|Figure 5: Plain X-ray of the abdomen with dilated bowel loops and air fluid level|
Click here to view
The infant was not responding to the conventional antibiotics and in view of the low socioeconomic status and lack of affordability, intravenous immunoglobulin could not be started and hence the child was started with ecosprin 80 mg/kg/day and conservatively managed with intravenous fluids. The infant gradually improved clinically over following 4 weeks.
| Discussion|| |
In our case, the presenting feature of fever, seizures, altered sensorium, paralytic ileus followed by rash led to the differential diagnosis of toxic shock syndrome (TSS) and Kawasaki disease. Both conditions present as fever with systemic features and add to the confusion.
In TSS, the rash is of scarlitiform pattern whereas our patient presented with perineal rash and erythema of palms and soles. The fever too was not responding to the antibiotics. Thus, the diagnosis was in favor of Kawasaki disease. There were no raised anti-streptolysin O titers or any other demonstrable foci of infection hence excluding the diagnosis of TSS. In staphylococcal scalded skin syndrome (SSSS), the child is toxic with extensive erythematous skin with blistering and erosions. However, the SSSS has a short course with resolution of disease within 1-2 weeks and responds to antibiotics.
The most common complication of Kawasaki disease are coronary artery abnormalities, but it may involve other organs. 
The etiology of Kawasaki disease remains obscure. Epidemiological evidence suggests that microbial agent is the likely cause. However, no causative organism has been identified. Recent hypothesis is that the disease is caused by a bacterial super antigen toxin similar to that responsible for TSS. 
There are no laboratory tests, which can help the clinician in arriving at the diagnosis of Kawasaki disease; however, the acute phase reactants such as the raised ESR, raised C-reactive protein and leucocytosis without foci of infection will contribute to the diagnosis.
The medical management of Kawasaki disease involves the use of gamma globulins and aspirin. Aspirin is continued in a low dose indefinitely if coronary artery abnormalities are present and if no evidence of abnormality is detected then it can be discontinued after 6-8 weeks.  Aspirin helps in reducing the risk of platelet aggregation.  In this case, immunoglobulins could not be started because of economical constraint of the patient, but the gradual improvement of the child could be explained with the resolution of the acute and subacute phase of the disease.
It can be concluded that Kawasaki disease should be kept as a possibility in a case of a child less than 5 years of age with fever of 5 or more days and not responding to antibiotic therapy. 
The aim of presenting this case is that:
- Disease is rarely seen in infants less than 6 months of age. So far the youngest case reported in Japan is an infant of 20 days. Infant of 1 month and 7 weeks were reported in Virginia. ,, Our case was an infant of 35 days and as per our knowledge the youngest reported from India
- Intestinal pseudo obstruction in Kawasaki disease is rare 
- Patient presented with multiple systemic manifestations-pseudo obstruction, aseptic meningitis and sterile pyuria, which are unusual associations ,
- Perineal rash presenting with vesicles is unusual. 
| Acknowledgment|| |
Department of Pediatrics, S. Nijalingappa Medical College and HSK Research Center, Bagalkot.
| References|| |
|1.||Leung DY, Lucky AW. Kawasaki syndrome. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Stephen IK, editors. Fitzpatrick's Dermatology in General Medicine. Vol. 2, 6 th ed. New York: Mc Graw Hill Publication; 2003. p. 1981-7. |
|2.||Kim MY, Noh JH. A case of Kawasaki disease with colonic edema. J Korean Med Sci 2008;23:723-6. |
|3.||Bowler J, Drvaric DM, Roberts JM, Burke SW. Kawasaki syndrome presenting as pyarthrosis of the hip. A case report. J Bone Joint Surg Am 1986;68:467-8. |
|4.||Pemberton MN, Doughty IM, Middlehurst RJ, Thornhill MH. Recurrent Kawasaki disease. Br Dent J 1999;186:270-1. |
|5.||Bhardwaj P, Sharma VK. Fever and rash: It can be Kawasaki disease. Indian J Dermatol 2009;54:29-316. |
|6.||Hay RJ, Adriaans BM. Bacterial infections. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Text Book of Dermatology. Vol. 2, 7 th ed. Oxford: Blackwell Publishing; 2004. p. 27.81. |
|7.||Tsuchida S, Yamanaka T, Tsuchida R, Nakamura Y, Yashiro M, Yanagawa H. Epidemiology of infant Kawasaki disease with a report of the youngest neonatal case ever reported in Japan. Acta Paediatr 1996;85:995-7. |
|8.||Durall AL, Phillips JR, Weisse ME, Mullett CJ. Infantile Kawasaki disease and peripheral gangrene. J Pediatr 2006;149:131-3. |
|9.||O'connor MJ, Saulsbury FT. Incomplete and atypical Kawasaki disease in a young infant: Severe, recalcitrant disease responsive to infliximab. Clin Pediatr (Phila) 2007;46:345-8. |
|10.||Tiao MM, Huang LT, Liang CD, Ko SF. Atypical Kawasaki disease presenting as intestinal pseudo-obstruction. J Formos Med Assoc 2006;105:252-5. |
|11.||Mancini AJ, Shani-Adir A. Other viral diseases. In: Bolognia JL, Jorizzo J, Rapini RP, editors. Dermatology. Vol. 2, 2 nd ed. Oxford: Elsevier Publication; 2009. p. 1232-3. |
|12.||Friter BS, Lucky AW. The perineal eruption of Kawasaki syndrome. Arch Dermatol 1988;124:1805-10. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]