Kawasaki Disease (KD), also known as mucocutaneous lymph node syndrome, is a rare but critical illness that predominantly affects children under the age of 5. It is a medium-vessel vasculitis with a particular predilection for coronary arteries, making early diagnosis and treatment essential to prevent long-term cardiovascular complications.
One of the most efficient ways to remember the diagnostic criteria of Kawasaki Disease is through the mnemonic "CREAM", which helps clinicians and medical students quickly recall the five major clinical features necessary for diagnosis. This article aims to decode the "CREAM" mnemonic, delve deep into each component, and offer a thorough understanding of the disease—its pathogenesis, symptoms, diagnosis, treatment, complications, and prognosis.
Understanding Kawasaki Disease
Kawasaki Disease is an acute febrile illness of early childhood that involves inflammation of the blood vessels, particularly coronary arteries. While its exact etiology remains unknown, it is believed to be triggered by an infectious agent in genetically predisposed individuals.
Epidemiology
- Most common in children under 5 years
- Boys are more frequently affected than girls
- Highest incidence reported in Japan
- Peak incidence: Winter and early spring
Pathophysiology
The disease is characterized by immune-mediated vasculitis. It affects medium-sized arteries, especially coronary arteries, leading to aneurysm formation, stenosis, or myocardial infarction in severe cases.
The hallmark features are fever and mucocutaneous manifestations.
Diagnostic Criteria: The "CREAM" Mnemonic
To make a clinical diagnosis of Kawasaki Disease, the following criteria must be met:
Fever for ≥5 days plus at least four of the following five features.
These five features are captured in the mnemonic CREAM:
| Letter | Stands For | Clinical Detail |
|---|---|---|
| C | Conjunctivitis | Bilateral, non-exudative conjunctivitis, typically painless and limbus-sparing |
| R | Rash | Polymorphous, non-vesicular rash involving trunk, extremities, and perineum |
| E | Edema or Erythema | Swelling or redness of hands and feet, often with later desquamation |
| A | Adenopathy | Cervical lymphadenopathy (>1.5 cm), usually unilateral |
| M | Mucosal Involvement | Strawberry tongue, red cracked lips, erythema of oral mucosa |
Let’s explore each of these signs in clinical detail.
C – Conjunctivitis (Non-exudative)
One of the earliest signs of Kawasaki Disease, conjunctivitis, typically appears within the first 2–4 days of fever onset. What distinguishes it from viral or bacterial causes is:
- Bilateral involvement
- No pus or discharge (non-exudative)
- Sparing of the limbus (the border between the cornea and sclera)
- Painless with minimal photophobia
This conjunctival injection indicates inflammation without the purulence typically seen in bacterial infections.
R – Rash (Polymorphous, Non-vesicular)
The rash in Kawasaki Disease is highly variable but generally fits the following description:
- Polymorphous: Can be maculopapular, urticarial, or resemble erythema multiforme
- Non-vesicular: No blisters or pustules
- Widespread: Trunk, limbs, groin, and perineal area
- Desquamation: Especially around the perineum, may occur later in the illness
Importantly, the rash is not pruritic, which helps differentiate it from allergic reactions or viral exanthems.
E – Edema or Erythema of Hands and Feet
This sign is particularly helpful in early diagnosis and evolves through three stages:
- Acute phase: Erythema and induration of palms and soles
- Subacute phase: Painful edema and swelling
- Convalescent phase: Peeling or desquamation around the fingers and toes
This peripheral change is distinctive and often seen within the first week of illness.
A – Adenopathy (Cervical Lymphadenopathy)
Though this feature is the least commonly observed of the five, its presence is significant:
- ≥1.5 cm lymph node in diameter
- Usually unilateral
- Anterior cervical chain is most frequently involved
- Firm, non-suppurative, and often tender
Adenopathy in KD is typically isolated, without signs of systemic infection like pharyngitis or otitis.
M – Mucosal Involvement (Oral Changes)
The mucosal signs in Kawasaki Disease are among the most characteristic and are often quite striking:
- Strawberry tongue: Enlarged, red papillae with bright red background
- Erythematous and fissured lips
- Redness of oral and pharyngeal mucosa
- No vesicles or ulcers
These findings typically appear early in the disease and can persist into the subacute phase.
Additional Diagnostic Considerations
While the mnemonic CREAM encapsulates the principal features, it's critical to remember:
- Not all children will meet all five criteria.
- Incomplete Kawasaki Disease may occur, especially in infants.
- Fever is mandatory—persisting for at least 5 days.
- Coronary artery abnormalities on echocardiogram may substitute one clinical criterion in suspected incomplete cases.
Laboratory Findings in Kawasaki Disease
Though the diagnosis is clinical, certain labs can support the diagnosis:
| Parameter | Findings |
|---|---|
| WBC | Leukocytosis with neutrophilia |
| ESR/CRP | Elevated |
| Platelets | Thrombocytosis (after day 7) |
| LFTs | Mild transaminitis |
| Urine | Sterile pyuria |
| Echocardiogram | Coronary artery aneurysms or dilatation |
These lab values help reinforce the diagnosis and monitor systemic involvement.
Differential Diagnosis
Because Kawasaki Disease mimics many other conditions, it's important to differentiate it from:
- Scarlet fever (due to streptococcus)
- Measles
- Stevens-Johnson syndrome
- Toxic shock syndrome
- Systemic juvenile idiopathic arthritis
Key differentiators include the non-exudative conjunctivitis, strawberry tongue without vesicles, and lack of exanthem desquamation until later.
Complications of Kawasaki Disease
The most feared complication of KD is coronary artery involvement:
- Coronary artery aneurysms
- Myocarditis
- Pericardial effusion
- Arrhythmias
- Myocardial infarction
Timely treatment dramatically reduces these risks.
Treatment of Kawasaki Disease
1. Intravenous Immunoglobulin (IVIG)
- Dose: 2 g/kg IV over 10–12 hours
- Best given within the first 10 days of illness
- Dramatically reduces incidence of coronary artery aneurysms
2. Aspirin
- High-dose (anti-inflammatory): 30–50 mg/kg/day in 4 divided doses during acute phase
- Low-dose (anti-platelet): 3–5 mg/kg/day once fever subsides
- Continue until no coronary changes or longer if abnormalities persist
3. Corticosteroids
- For IVIG-resistant KD
- Especially useful in high-risk patients or refractory inflammation
Prognosis and Long-Term Management
With early treatment, the prognosis of Kawasaki Disease is excellent:
- Over 95% recover fully if treated within the first 10 days
- Coronary artery aneurysms develop in <5% of treated children
- Lifelong cardiology follow-up may be necessary in cases with vascular abnormalities
Echocardiography is repeated at 2 weeks and 6–8 weeks to monitor resolution or progression.
FAQ on Kawasaki Disease
Q1. What is the earliest symptom of Kawasaki Disease?
Fever that persists beyond 5 days is the earliest and most consistent sign.
Q2. Is Kawasaki Disease contagious?
No, it is not directly contagious. It is suspected to be triggered by infectious agents, but the disease itself is not spread person to person.
Q3. Can adults get Kawasaki Disease?
It is extremely rare in adults. The condition primarily affects children under the age of 5.
Q4. How is Kawasaki Disease different from measles or scarlet fever?
KD features non-exudative conjunctivitis, no vesicles, and prominent limb changes not typically seen in measles or scarlet fever.
Q5. What if IVIG doesn’t work?
In IVIG-resistant cases, corticosteroids or infliximab (anti-TNF) may be used.
Q6. Can KD recur?
Yes, although rare, recurrence is possible and typically occurs within two years.
Q7. What does CREAM stand for again?
- C – Conjunctivitis
- R – Rash
- E – Edema/Erythema of extremities
- A – Adenopathy
- M – Mucosal changes
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