Kawasaki disease

Background

• Also known as: mucocutaneous lymph node syndrome
• Vasculitis of unknown etiology
• Peaks at 18-24 months
  • Rare in <4mo, >5yr
• Leading cause of acquired heart disease in children
• Coronary aneurysm more common in incomplete than in classic KD

Clinical Features

• Fever that is high, abrupt
• Rash often seen in perineum; accompanies onset of fever
  • Maculopapular most common; vesicles not seen
• Cardiac complications develop early on
  • Coronary artery aneurysm development most prevalent as fever lessens

Associated Symptoms

• Cardiac
  • Coronary aneurysm
    • Most develop during 3-4th week of illness
    • May lead to MI (leading cause of death)
  • Myo/pericarditis
  • Pericardial effusion
  • LV dysnfunction
  • Valvular dysfunction
  • Dysrhythmias
• Labs
  • Elevated ESR/WBC/LFTs/Plts
• Aseptic meningitis
• Urethritis
• Anemia
• RUQ pain, large GB (hydrops on US)

Differential Diagnosis

Pediatric fever

• Upper respiratory infection (URI)
• UTI
• Sepsis
• Meningitis
• Febrile seizure
• Juvenile rheumatoid arthritis
• Pneumonia
• Acute otitis media
• Whooping cough
• Unclear source
• Kawasaki disease
• Neonatal HSV
• Specific virus
  • COVID-19 (peds)
    • MIS-C

Erythematous rash

• Positive Nikolsky’s sign
  • Febrile
    • Staphylococcal scalded skin syndrome (children)
    • Toxic epidermal necrolysis/SJS (adults)
  • Afebrile
    • Toxic epidermal necrolysis
• Negative Nikolsky’s sign
  • Febrile
    • Toxic shock syndrome
    • Kawasaki disease
    • Scarlet fever
    • DRESS syndrome
    • Cellulitis
    • Acute generalized exanthematous pustulosis
  • Afebrile
    • Alcohol intoxication
    • Anaphylaxis
    • Cellulitis
    • Dermatitis (multiple types)
    • Drug rash
    • Erysipelas
    • Erythema multiforme
    • Exfoliative erythroderma
    • Scombroid
    • Vancomycin infusion reaction

Evaluation

Work-Up

• CBC
• LFTs
• ESR, CRP
• Blood culture
• UA
• ECG
• TTE (Coronaries, LV, valves)
• Red Top "Kawasaki Serum to CBR"

Evaluation

• Clinical diagnosis

Classic Kawasaki Disease	Fever for 5 days or more plus four of the following symptoms
	1. Bilateral nonexudative conjunctivitis
	2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosae)
	3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage)
	4. Rash
	5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical)
Incomplete Kawasaki Disease	Fever for 5 days and two to three clinical criteria of classic Kawasaki disease plus
	C-reactive protein 3.0 milligrams/L and/or erythrocyte sedimentation rate 40 mm/h plus positive echocardiogram or three or more of the following:
	1. Albumin <3 grams/dL
	2. Anemia
	3. Elevated alanine aminotransferase (ALT)
	4. Platelets >450,000/mm3 7d after fever onset
	5. White blood cell count >12,000/mm3
	6. Pyuria

Management

• IVIG 2gm/kg over 12hr
• Aspirin 20mg/kg/dose q6h

Disposition

• Admit