Kawasaki disease

What it is

Kawasaki disease is a generalized vasculitis of unknown etiology, a medium-vessel vasculitis, with inflammation of primarily small to medium size arteries, which causes multisystem involvement. Among the vasculitides it sits with polyarteritis nodosa among the medium vessel vasculitides, apart from the small vessel and large vessel groups. It is predominately seen in children: 90% are under 5 years of age, and the incidence is increased in East Asian ethnicity. The trigger is given as likely viral.

How it presents

The fever is high-grade, lasts more than 5 days, and is characteristically resistant to antipyretics. With it come:

• Conjunctivitis that is bilateral and nonexudative, with bulbar conjunctival injection.
• Oropharyngeal mucositis: injected, fissured, bright red cracked lips, and a strawberry tongue, red and swollen with prominent papillae.
• Cervical lymphadenopathy, often unilateral, with at least one node over 1.5 cm in diameter.
• A rash that is erythematous, polymorphous, non-vesicular and generalized, with perineal erythema and desquamation, and a morbilliform rash on the trunk and extremities.
• Erythema and edema of the hands and feet, with red palms and soles which later peel, and desquamation of the fingertips.

The child is irritable as well; the picture in a 2-year-old runs as high grade fever for 5 days with bilateral congested conjunctivae, cervical lymphadenopathy, cracked lips and swelling of both hands and feet.