Clinical Manifestations

The clinical findings in acute KD evolve over the first week after onset of fever and include the features described in Table 1. The onset of fever is usually abrupt, without a prodrome, and has a spiking/remitting pattern throughout the day. More than 90% of patients develop bilateral, symmetrical dilatation of vessels in the bulbar conjunctiva with limbal sparing, which creates a "halo effect" around the iris (24). There is no exudate, and conjunctival biopsy reveals no local inflammatory response (25).

Changes of the lips and oral cavity include dryness, erythema and fissuring of the lips, diffuse erythema of the oropharynx without pharyngeal exudates or discrete intraoral lesions, and a strawberry tongue with sloughing of the filiform papillae with prominence of the fungiform and circumvallate papillae (26). Hoarseness is frequently noted and direct fiberoptic laryngoscopy has revealed marked edema and erythema of the vocal cords (Dr. Deborah Don, Rady Children's Hospital of San Diego, personal communication).

The rash is a polymorphous exanthem with nonspecific changes on biopsy (27). Target lesions, petechiae, and micropustules may be seen, but the rash is never frankly bullous, thus differentiating it from Stevens-Johnson syndrome (Chapter 22) (1,28). The rash may be dramatically accentuated in the perineum in over 50% of patients (29).

Changes in the extremities may include erythema of the palms and soles, indurative edema of the dorsa of the hands and feet, and periungual desquamation during the subacute phase (second to third week of illness). Not uncommonly, the diagnosis of KD is made retrospectively when the characteristic peeling of the fingers and toes is noted. Unfortunately, coronary artery damage from the vasculitis may already be apparent at this point in the illness.

Although unilateral or bilateral, nonsuppurative cervical lymphadenopathy with at least one node measuring at least 1.5 cm was among the original criteria described by Kawasaki, it has been noted in only 40% to 50% of patients in more recent series (1,26,30). In one series, 43 of 83 KD patients (52%) developed cervical adenopathy during their acute illness and 18 of these 43 patients (42%) were initially misdiagnosed and treated for bacterial cervical lymphadenitis (30). Patients presenting with cervical adenopathy tended to be older and have a higher erythrocyte sedimentation rate and total white blood cell count than KD patients who never developed clinically apparent cervical node enlargement. In another series of 50 KD patients from Canada, 16 of the 50 patients (32%) presented with fever and a prominent otolaryngologic condition (cervical adenitis, tonsillitis, or otitis media) that misled the treating physician and delayed the correct diagnosis of KD (31). Initial misdiagnosis is common and 60% to 90% of KD patients in various series were initially treated with antibiotics before the correct diagnosis was made (32,33). The otolaryngologist should consider the possibility of KD in any pediatric patient who is not responding to antibiotic treatment for presumed bacterial cervical lymphadenitis (33-36). Case reports have documented the association of KD with retropharyngeal soft-tissue swelling, mastoiditis, and upper airway compromise (34,37-45).

Other clinical and laboratory features that may accompany the systemic vasculitis of KD include arthralgia and arthritis, hydrops of the gallbladder with associated abdominal pain and elevated g glutamyltranspeptidase, sterile pyuria, anterior uveitis, meningitis with cerebrospinal fluid (CSF) pleocytosis and irritability, and sensorineural hearing loss (26,46,47). Case reports from the United States and Japan have documented 20 patients with moderate-to-profound, permanent sensorineural hearing loss following the acute phase of KD (48,49). A prospective, multicenter study conducted in North America documented sensorineural hearing loss (20-35 dB) in 19 of 62 patients (30%) evaluated within 30 days after onset of fever using visual reinforcement, play audiometry, tympanometry, and brainstem auditory-evoked response testing in patients below one year of age or in patients for whom the results of behavioral audiometry were deemed unreliable (50). Persistent hearing loss was noted in 2 of 36 patients (5.5%) evaluated at least 10 days after the first evaluation. The investigators concluded that routine audiologic screening of KD patients was not warranted but that health-care providers should be aware of the possibility of profound sensorineural hearing loss as a rare complication of KD. The pathogenesis of hearing loss in this setting is unknown, although direct cytopathic effect of the causative agent in the cochlea or labyrinth, vasculitis of the vasonervorum of cranial nerve VIII, or direct immunologic attack on components of the inner ear have been proposed (50). Sensorineural hearing loss is discussed in detail in Chapter 28.

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