What is the diagnosis and treatment for KPV (Keratoacanthoma Pilonatrixoma Verrucous) or Kawasaki disease?

Diagnosis and Treatment of Kawasaki Disease

Kawasaki disease is diagnosed by the presence of fever lasting at least 5 days plus 4 out of 5 principal clinical features, and treatment consists of intravenous immunoglobulin (IVIG) 2 g/kg as a single infusion along with high-dose aspirin (80-100 mg/kg/day divided into four doses). 1

Diagnostic Criteria for Kawasaki Disease

Principal Clinical Features

1. Fever: High-spiking (>39°C/102.2°F), remittent, persisting for at least 5 days without treatment 2
2. Changes in the oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse erythema of oral and pharyngeal mucosa (without exudates or ulcerations) 2, 1
3. Bilateral bulbar conjunctival injection: Non-exudative, often sparing the limbus 2, 1
4. Polymorphous rash: Usually appearing within 5 days of fever onset, primarily truncal with groin accentuation 2, 1
5. Changes in extremities: Erythema and edema of hands/feet in acute phase, periungual desquamation in convalescent phase 2, 1
6. Cervical lymphadenopathy: Usually unilateral, ≥1.5 cm in diameter, confined to anterior cervical triangle 2, 1

Incomplete (Atypical) Kawasaki Disease

• Consider in any child with prolonged unexplained fever (≥5 days) with 2-3 principal features 2
• More common in infants <6 months, who are at high risk for coronary artery abnormalities 2
• Laboratory findings and cardiovascular sequelae are the same as in complete cases 2

Laboratory Findings

• Elevated ESR and CRP
• Leukocytosis with neutrophil predominance
• Hypoalbuminemia
• Mild anemia
• Thrombocytosis (typically in second week)
• Sterile pyuria
• Elevated liver enzymes 2, 1

Treatment Algorithm

Initial Treatment

1. IVIG: 2 g/kg as a single infusion, administered within 10 days of illness onset 2, 1

   • Reduces risk of coronary artery aneurysms from 20-25% to <5%
   • Should be given even after 10 days if fever persists or coronary abnormalities are present

2. Aspirin:

   • Acute phase: High-dose (80-100 mg/kg/day divided into four doses) 1
   • Continue until patient is afebrile for 48-72 hours
   • Then reduce to low-dose (3-5 mg/kg/day) for antiplatelet effect 1

Treatment of IVIG-Resistant Cases

For patients with persistent or recurrent fever within 36 hours after completing initial IVIG:

1. Second dose of IVIG: 2 g/kg as a single infusion 2, 1

2. If fever persists after second IVIG dose:

   • Corticosteroids: Methylprednisolone 20-30 mg/kg IV for 3 days, with or without oral prednisone taper 2
   • Infliximab: 5 mg/kg as a single infusion (alternative to second IVIG or corticosteroids) 2
   • Cyclosporine: Consider when other therapies have failed 2
   • Other options for highly refractory cases: IL-1 blockers (anakinra), plasma exchange, or cytotoxic agents 2

Cardiac Evaluation and Monitoring

Initial Echocardiography

• Should be performed when KD is suspected 2
• Assess coronary arteries with quantitative measurements of luminal dimensions
• Calculate Z scores adjusted for body surface area (Z score ≥2.5 indicates abnormality) 2

Follow-up Echocardiography

• For uncomplicated patients: Repeat within 1-2 weeks and 4-6 weeks after treatment 2
• For patients with coronary abnormalities (Z score >2.5): More frequent imaging (at least twice weekly) until dimensions stabilize 2

Common Pitfalls in Diagnosis

1. Misdiagnosis in infants: Prolonged fever and irritability may be the only clinical manifestations in infants <6 months 2

2. Delayed diagnosis in older children/adolescents: May present with fewer classic symptoms but have high prevalence of coronary abnormalities 2

3. Confusion with other conditions:

   • Fever and pyuria misdiagnosed as urinary tract infection
   • Rash development after antibiotics misinterpreted as drug reaction
   • Cervical lymphadenopathy misdiagnosed as bacterial lymphadenitis 2
   • Fever and cerebrospinal fluid pleocytosis misdiagnosed as viral meningitis 2

4. Incomplete recognition of symptoms: Clinical features may not all be present simultaneously, and some may have resolved by the time of presentation 2

Long-term Management

Management is based on the degree of coronary involvement:

• No coronary abnormalities: Low-dose aspirin for 6-8 weeks
• Small-to-medium aneurysms: Continued antiplatelet therapy and regular cardiac monitoring
• Large or giant aneurysms: Antiplatelet therapy, anticoagulation, and more intensive monitoring 1

KD is now the leading cause of acquired heart disease in children in many developed countries, making prompt diagnosis and treatment essential for preventing long-term cardiac complications 3.