Journal of Postgraduate Medicine Medknow Publications and Staff Society of Seth GS Medical College and KEM Hospital, Mumbai, India ISSN: 0022-3859 EISSN: 0972-2823 Vol. 49, Num. 1, 2003, pp. 72-74

Journal of Postgraduate Medicine, Vol. 49, No. 1, Jan-March, 2003, pp. 72-74

Incomplete Kawasaki Disease with Recurrent Skin Peeling: A Case Report with the Review of Literature

Parmar RC, Somale A, Bavdekar SB, Muranjan MN

Department of Paediatrics, Seth G. S. Medical College and K. E. M. Hospital, Parel, Mumbai - 400 012, India.
Address for Correspondence: Ramesh Parmar, MD, Department of Paediatric Cardiology, Madras Medical Mission Hospital, 4A, JJ Nagar, Moggapair, Chennai-600050. E-mail: rameshparmar@yahoo.com Tabl

Code Number: jp03013

Abstract:

Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a five-year-old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. During the six-month follow up period, she had two episodes of recurrent skin peeling a phenomenon, which is recently reported with KD but not with atypical or incomplete KD. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a "high index of suspicion" for KD and even, higher for IKD. (J Postgrad Med 2003;49:72-74)

Key Words: Incomplete Kawasaki disease, Vasculitis, Thrombocytosis, Intravenous immunoglobulin, Skin peeling.

Kawasaki Disease (KD) is an acute systemic vasculitis of unknown aetiology with a variable incidence. In Japan, the incidence is 80-100 per 100,000 children under five years of age, 8 in the USA, 3-6 in Europe.^1-3 Despite such high incidence world over, the number of reported cases from India remains meagre.^4-6 In recent years, patients have been described who did not fulfil the clinical criteria for the diagnosis of KD but nevertheless, went on to develop coronary abnormalities classically seen in patients with KD.^7,8 These were labelled as incomplete or atypical KD.

We report a case of incomplete KD (IKD), rarely reported in Indian literature, with associated recurrent skin peeling [a phenomenon recently reported in patients with KD but not with IKD].^9,10

Case Report

A five-year-old girl presented with redness of the tongue and desquamation of palms and soles for six days and high-grade fever of two days. There was no history suggestive of specific focus of infection, rash, diarrhoea, headache, convulsion, altered sensorium, arthralgia, chest pain, palpitations or syncopal attacks or drug intake.

Examination revealed a febrile child with significantly enlarged post-auricular, jugulo-diagastric and submandibular lymph nodes. The lips were dry, cracked and red and the tongue was strawberry red. There was periungual desquamation over the fingers. The skin over the dorsum of feet showed fine scaling. There was striking erythema confined to the palms and soles, with an abrupt change to normal-appearing skin at the wrists and ankles.

Investigations revealed total WBC count of 13,600/mm³ with 70% neutrophils, elevated ESR (60 mm at the end of 1 hour) and raised serum C-reactive protein (96 mg/l), and normal platelet count (1,80,000/mm³). Antistreptolysin-O titres were normal. Culture of throat swab did not reveal any growth. Weil-Felix test was negative. Serum transaminases were not elevated. Urine examination and culture study demonstrated sterile pyuria. Tests for the detection of anti-nuclear antibodies and antibodies against double-stranded DNA were negative. A diagnosis of KD was considered. However, the absence of rash and conjunctival injection made the diagnosis suspect. Absence of conjunctival vessel dilatation and iridocyclitis was confirmed by slit lamp examination. Exclusion of diseases with similar presentation was made (Table 1). A diagnosis of IKD was made as per the Japanese worker's criteria.¹¹ Raised acute phase reactants and sterile pyuria gave additional support to the diagnosis. Chest radiograph, ECG and echocardiography were normal.

She was treated with a single dose of intravenous immunoglobulin (IVIG, 2 gm/kg over ten hours) and aspirin (80 mg/kg/day for 7 days followed by 5 mg/kg/day for the next 6 weeks). She did not develop any additional clinical abnormalities and the fever subsided within a day. The platelet count on day 10 was 800,000/mm.³ Echocardiography done in the 7^th week did not reveal any abnormality of the coronary vasculature.

Clinical and echocardiographic evaluations done up to 6 months were normal. She developed two episodes of recurrent peeling of the skin during her 6-month follow-up period for which no obvious cause could be found. They were not related to environmental changes. The peeling episodes occurred spontaneously over hands and feet and lasted for a period of one week each and disappeared without treatment.

Discussion

After Henoch-Schonlein purpura, KD is the commonest vasculitic disorder of children.^1-6 Albeit the number of cases reported from India remains minuscule and is largely confined to typical KD. It is likely that many cases of KD and more so of IKD go unnoticed, especially so in the absence of a precise diagnostic test.

Diagnosis of KD is based on clinical criteria (Table 1); five of the six criteria are considered essential for the diagnosis.¹¹ These features need not be present at one particular time and in fact, may evolve sequentially over a period of few days. Diagnostic difficulties arise when only a subset of manifestations occurs, as happened in our case. The four diagnostic clinical criteria (Table 1) in our patient satisfied the Japanese worker's criteria^8,12 for the diagnosis of IKD. The patient had high levels of acute phase reactants and sterile pyuria, and subsequently went on to develop thrombocytosis during convalescence, which are known to occur in KD.

While skin peeling after an acute episode of KD is common, our patient developed recurrent episodes of skin peeling, a phenomenon noticed in 11% cases with KD,¹⁰ but hitherto not with IKD. Physicians need to be aware of this phenomenon, which is distinct from recurrence of Kawasaki disease. Repeeling may persist for several years after an episode of KD before resolving with no long-term sequel. The areas of skin, which undergo repeeling, are seldom the most severely affected areas during the first episode of KD. With a few exceptions, the patients with repeeling are the ones who do not develop measurable coronary artery dilatation and in fact, have a milder course of the illness as in our case. Repeeling of skin does not seem to correlate to the timing of the commencement of therapy with immunoglobulin, use of aspirin or the timing of hospitalisation. Its mechanism remains unclear. Children with notable behavioural sequelae or hearing problems following KD were observed at a higher frequency in the cohort, which had recurrent desquammation.¹⁰

Diagnosing classical KD is difficult enough. Recognising cases that do not fully meet the syndrome diagnostic criteria offers an even greater challenge. This is only the second case of IKD reported in the Indian literature.⁹ Nevertheless it is important to be aware of this entity, as an unaware clinician may face a diagnostic dilemma. On one hand, there is just not enough clinical evidence for diagnosing KD. On the other hand, waiting endlessly could lead to irreversible complications such as coronary artery aneurysms and subsequent propensity to develop cardiac problems. In "incomplete" or "atypical" forms all the essential manifestations are not present. To help clinicians to arrive at the diagnosis, Japanese and North American groups have evolved diagnostic criteria.^8,12 The Japanese workers deem presence of any four of the six clinical criteria sufficient for the diagnosis of IKD while the North American workers consider the presence of three criteria to be sufficient for the diagnosis of IKD provided echocardiography or angiography reveals coronary artery changes due to arteritis. As coronary lesions are often present by day 9 or 10 of illness, various workers have attempted to find out if any of the laboratory criteria could help in diagnosing atypical or incomplete forms of KD.¹³ Levy et al¹⁴ found that thrombocytosis was present consistently in atypical forms with peak thrombocytosis occurring at 13.5±5.9 days of illness and concluded that presence of thrombocytosis should be considered compatible with the diagnosis of IKD. Albeit waiting for detecting thrombocytosis and coronary lesion to confirm the diagnosis of IKD can adversely affect the prognosis. For prevention of the complications, the physician has to start therapy much earlier. Hence, it is imperative that one diagnoses KD early and institutes treatment promptly. This is true of IKD too as this form has the tendency to develop all the complications of the classic KD.^7,8 Unless a high index of suspicion is kept the patients with IKD will continue to escape our attention.

References

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11. Japan Kawasaki Disease Research Committee. Diagnostic guidelines of Kawasaki disease, 4^th Revision. Tokyo: Kawasaki Disease Research Committee; 1984.
12. Witt MT, Luann ML, Bohnsack JF, Young PC. Kawasaki disease: more patients are being diagnosed who do not meet American Heart Association criteria. Pediatrics 1999;104:e10.
13. Dengler DL, Capparelli EV, Bastian JF, Bradley DJ, Glode MP, Santa S, et al. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J 1998;17:478-81.
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