INTRODUCTION

Kawasaki disease (KD, also called mucocutaneous lymph node syndrome) is one of the most common vasculitides of childhood [1]. It is typically a self-limited condition, with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy. However, complications such as coronary artery aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, arrhythmias, and peripheral arterial occlusion may develop and lead to significant morbidity and mortality. (See "Cardiovascular sequelae of Kawasaki disease".)

The frequencies of aneurysm development and mortality have been dramatically decreased as a result of intravenous immune globulin (IVIG) therapy [2]. Expeditious diagnosis is critical to achieve the optimal treatment result.

The clinical manifestations and diagnosis of KD, including incomplete (atypical) KD will be discussed in this review. The epidemiology, etiology, treatment, and cardiac sequelae of Kawasaki disease are presented separately. (See "Epidemiology and etiology of Kawasaki disease" and "Initial treatment and prognosis of Kawasaki disease" and "Cardiovascular sequelae of Kawasaki disease".)

CLINICAL MANIFESTATIONS

KD is characterized by systemic inflammation manifested by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, rash, extremity changes and lymphadenopathy. These clinical signs are the basis for the diagnostic criteria for KD (table 1). These findings are often not present at the same time. As an example, the only clinical features some patients have developed by the time of admission are fever and cervical lymphadenopathy (KD with isolated cervical lymphadenopathy, KDiL) [3]. In one case series, these patients tended to be older and have a more severe course, with increased risk of coronary artery disease and lack of response to intravenous immune globulin. Thus, repeated histories and physical examinations are important in making a timely diagnosis of KD. (See 'Diagnosis' below.)

Fever — Fever is the most consistent manifestation of KD. It reflects elevated levels of proinflammatory cytokines such as tumor necrosis factor and interleukin-1, which are thought to mediate the underlying vascular inflammation [4]. Fever is minimally responsive to antipyretic agents, and remains above 38.5ºC during most of the illness. Patients are often described as irritable. The diagnosis of KD should be considered in all children with prolonged unexplained fever ≥5 days. (See 'Incomplete (atypical) KD' below.)