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CASE REPORT
Year : 2009  |  Volume : 54  |  Issue : 5  |  Page : 29-31
Fever and rash: It can be Kawasaki disease


Department of Pediatrics, Indira Gandhi Medical College, Shimla, HP, India

Correspondence Address:
Parveen Bhardwaj
Department of Pediatrics, Indira Gandhi Medical College, Shimla - 171 001, HP
India
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Source of Support: None, Conflict of Interest: None


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   Abstract 

Kawasaki disease (KD) is an illness that involves the skin, mouth, and lymph nodes, and typically affects children who are under the age of five. The cause of KD is unknown, but if the symptoms are recognized early, children with the disease can fully recover within a few days. A generalized vasculitis of unknown etiology, early recognition and treatment of KD can reduce the development of potentially life-threatening coronary artery abnormalities.


Keywords: Coronary aneurysm, Kawasaki disease, vasculitis


How to cite this article:
Bhardwaj P, Sharma VK. Fever and rash: It can be Kawasaki disease. Indian J Dermatol 2009;54, Suppl S1:29-31

How to cite this URL:
Bhardwaj P, Sharma VK. Fever and rash: It can be Kawasaki disease. Indian J Dermatol [serial online] 2009 [cited 2019 Oct 18];54, Suppl S1:29-31. Available from: http://www.e-ijd.org/text.asp?2009/54/5/29/45439



   Introduction Top


Kawasaki disease (KD) or syndrome is a generalized vasculitis of unknown etiology and a leading cause of acquired heart disease among children living in developed countries, but is still is enigmatic in India, in the sense that it is diagnosed less often than it exists, reason may be there is no laboratory test which can help the clinician in arriving at or confirming a diagnosis of KD. The difficulty is further compounded by the fact that the clinical features gradually evolve over a period of days to a few weeks, and the entire clinical spectrum is not seen at any one particular point of time. Moreover, it may be difficult to distinguish KD from other common febrile illnesses of childhood and is usually misdiagnosed as viral exanthem more so due to lack of awareness among physicians and dermatologists. Since untreated cases may have coronary abnormalities, including coronary aneurysms in about one fourth of the cases and may be fatal if not treated. Hence we are reporting three cases of KD.


   Case History Top


We diagnosed three cases of KD. All these cases presented with fever, rash and were being treated with antibiotics from outside without any relief. The typical rash, strawberry tongue and desquamation in case 3 are shown in [Figure 1] (Desquamation and edema hand) and [Figure 2] (Rash and strawberry tongue). [Table 1] and [Table 2] describes Clinical and Investigational profile of the cases.

All the cases were treated with Intravenous Immunoglobulin (2gm/kg) infusion, case 3 required one additional dose of IVIG since fever persisted after first dose and Aspirin (100mg/kg) till 14 th day of illness and then 5mg/kg for the next six weeks. Echocardiography was done at diagnosis, after 2 nd week and 6 th week and all were normal.


   Discussion Top


First described in 1967 by Kawasaki, this illness was originally referred to as "mucocutaneous lymph node syndrome". [1] Kawasaki disease has been reported throughout the world. KD is by no means a rare disorder, with the reported annual incidence being in the range of 10-100 per 100,000 children below 5 years of age. [2] It is being increasingly reported from different parts of India. [3],[4] Kawasaki disease occurs more often in boys than in girls (ratio of about 1.5:1). Approximately 80 percent of affected children are less than five years old. Patient may present to pediatricians, otolaryngologist and dermatologists but still the cases are under reported, the main reason for under reporting is that usually it is misdiagnosed as viral exanthem and remains undiagnosed because of lack of awareness among physicians.

The etiology is unknown, although many suspect an infectious etiology. Even though the initiating event has not yet been identified, the immune system is known to be involved during the acute stage of Kawasaki disease.

In the absence of a specific diagnostic test, Kawasaki disease is a clinical diagnosis based on the characteristic history and physical findings. [5],[6] The diagnosis of classic KD requires fever of at least 5 days duration and the presence of 4 of the following:

  1. 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 [Figure 1].
  2. Bilateral conjunctivitis (not associated with exudates)
  3. Polymorphous rash (not vesicular) [Figure 2].
  4. Cervical lymphadenopathy (usually>1.5cm and unilateral; the least common of all clinical features, occurring in approximately 40%).
  5. Changes in the lips and oral cavity (e.g. pharyngeal erythema, dry/fissured or swollen lips, strawberry tongue) [Figure 2].


Marked irritability out of proportion to the degree of fever is usually present which responds dramatically to treatment. All of these were present in our cases.

However, "atypical" or "incomplete" cases of Kawasaki disease, in which patients have fewer than four of the five principal features, have been increasingly reported. Patients with fever and three of the principal clinical features can be diagnosed with Kawasaki disease when coronary artery disease is detected by two-dimensional echocardiography or coronary angiography.

The course of Kawasaki disease can be divided into three clinical phases: acute, sub acute and convalescent. The acute febrile phase which usually lasts seven to 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. The sub acute phase covers the period from the end of the fever to about day 25. During this phase, patients may have desquamation of the fingers and toes (most characteristic for Kawasaki disease), arthritis and arthralgia, and thrombocytosis. The convalescent phase begins when clinical signs disappear and continues until the erythrocyte sedimentation rate becomes normal, usually six to eight weeks after the onset of illness.

Cardiovascular manifestations can be prominent in the acute phase of the illness and are the leading cause of morbidity and mortality. Pancarditis may occur, and the coronary arteries may be affected. Coronary artery abnormalities develop in 20 to 25 percent of children with untreated Kawasaki disease. Abnormalities may include diffuse ectasia or coronary aneurysms. Aneurysms usually become apparent one to three weeks after the onset of fever; their appearance more than five weeks after the onset of illness is uncommon. Echocardiograms and angiograms obtained in long-term follow-up studies indicate that coronary aneurysms resolve within five to 18 months in approximately 50 percent of patients. [7]

It is of the utmost importance to diagnose KD in the acute stage (preferably in first 10 days) because as many as 20-30% of untreated patients may develop coronary artery abnormalities. With treatment this figure can be brought down to as low as 1-2%. [8]

Differential diagnosis of Kawasaki disease includes scarlet fever, toxic shock syndrome, measles, drug hypersensitivity reactions including  Stevens-Johnson syndrome More Details, juvenile rheumatoid arthritis, and leptospirosis. Some features of uncomplicated measles that help to distinguish it from Kawasaki disease include the presence of exudative conjunctivitis, Koplik spots, rash that begins on the face behind the ears, and a low white blood cell count and ESR. Some features of drug reactions, such as the presence of periorbital edema, oral ulcers, and a low ESR, may help to distinguish these reactions from Kawasaki disease. Toxic shock syndrome may be distinguished by the presence of hypotension, renal involvement, elevated creatine phosphokinase level, and a focus of Staphylococcus aureus infection. A common clinical problem is the differentiation of scarlet fever from Kawasaki disease in a child who is a group A streptococcal carrier. Because patients with scarlet fever have a rapid clinical response to penicillin therapy, such treatment for 24-48 hrs with clinical reassessment at that time generally clarifies the diagnosis. The presence of lymphadenopathy, hepatosplenomegaly, and an evanescent, salmon-colored rash suggests a diagnosis of juvenile rheumatoid arthritis.

There is no laboratory test which can help the clinician in arriving at or confirming a diagnosis of Kawasaki disease, however, acute phase reactants such as the ESR, CRP, and serum alpha1-antitrypsin level are elevated. A mild-to-moderate normochromic anemia and leucocytosis is observed in the acute stage. Culture results are all negative. During the sub acute stage, platelet count is elevated. In the convalescent stage, the levels of platelets and other markers begin to return to values within the reference range. Laboratory values may require 6-8 weeks to normalize.

The medical management of Kawasaki disease (KD) involves the use of gamma globulin and aspirin as anti-inflammatory agents and long-term anticoagulation. Aspirin can be discontinued after six to eight weeks if all echocardiograms show no evidence of coronary artery abnormalities. If coronary artery abnormalities are detected, low-dose aspirin therapy should be continued indefinitely.

So it can be concluded that Kawasaki disease should be kept as a possibility in a case of fever of more than 5 days and 4 out of 5 clinical symptoms or signs as mentioned previously especially in children less than 5 yrs of age, which is not improving after antibiotic therapy and after other causes for fever are ruled out.

 
   References Top

1.Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children. Arerugi 1967;16:178-222.  Back to cited text no. 1  [PUBMED]  
2.Kawasaki T. General review and problems in Kawasaki Disease. Jpn Heart J 1995;36:1-12.  Back to cited text no. 2  [PUBMED]  
3.Paul DK, Gupta A, Lahiri M. Kawasaki disease in Calcutta. Indian Pediatr 2000;37:1264-5.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.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. 4  [PUBMED]  
5.Japan Kawasaki Disease Research Committee. Diagnostic guideline of Kawasaki disease. Tokyo: Japan Kawasaki Disease Research Committee; 1984.  Back to cited text no. 5    
6.American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. Diagnostic guidelines for Kawasaki disease. Am J Dis Child 1990;144:1218-9.  Back to cited text no. 6  [PUBMED]  
7.kato H, Ichinose E, Yoshioka F, Takechi T, Matsunga S, SuzukiK, et al . Fate of coronary aneurysms in Kawasaki disease: Serial coronary angiography and long-term follow-up study. Am JCardiol 1982;49:1758-66.  Back to cited text no. 7    
8.Shulman ST, Bass JL, Bierman F, Burns JC, Chung K, DillonMJ, et al . Management of Kawasaki syndrome: A consensus statement prepared by North American participants of the Third International Kawasaki Disease Symposium, Tokyo, Japan, December 1988. Pediatr Infect Dis J 1989;8:663-7.  Back to cited text no. 8    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]



 

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    Abstract
    Introduction
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