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medU | Instructors

Case 11
Author: Robert Drucker, M.D., Duke University School of Medicine

Learning Objectives
1. Identify the features needed to make a diagnosis of Kawasaki disease.
2. Discuss the acute management of Kawasaki disease.
3. List complications associated with Kawasaki disease.
4. Recognize common viral exanthems.
5. Develop a differential diagnosis for fever and rash.
6. Develop a differential diagnosis for unilateral cervical adenopathy.

Summary of clinical scenario: Five-year-old Jason presents with a 104

Fahrenheit fever of seven days duration, a bumpy rash, conjunctivitis, irritability,
and refusal to walk. Physical exam reveals mucosal changes, unilateral cervical
adenopathy, maculopapular rash, and changes in his extremities. Based on history
and exam findings, Jason is diagnosed with Kawasaki disease and admitted for

Persistent fever
Key Findings from History Rash
Refusal to walk

Maculopapular rash
Nonpurulent conjunctivitis
Key Findings from
Mucosal changes
Physical Exam
Unilateral cervical adenopathy
Swelling in extremities

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Stevens-Johnson syndrome
Juvenile rheumatoid arthritis
Kawasaki disease
Differential Diagnosis
Rocky Mountain spotted fever
Scarlet fever

White blood count: Elevated, with a

predominance of neutrophils
Hemoglobin/hematocrit and red
Key Findings from Testing blood cell smear: Normochromic,
normocytic anemia
Mean corpuscular volume: Normal
Platelets: Normal

Final Diagnosis Kawasaki disease

Case highlights: The case teaches which lab tests to order to narrow the
differential diagnosis and how to manage Kawasaki disease and its complications
acutely and long-term. Multimedia highlights include photos of rashes and other
manifestations of several viral exanthems and echocardiograms showing both a
normal heart and one with a coronary aneurysm.

Key Teaching Points


Enterovirus: Rash usually erythematous and maculopapular and may involve the
palms and soles. In hand-foot-and-mouth disease (caused by Coxsackievirus),
there is a vesicular rash on hands and feet, with ulcers in the mouth.

Measles: Initial rash appears on buccal mucosa as red lesions with central bluish
white spots (Koplik spots). After prodromal fever (over 101 Fahrenheit), cough,
coryza, and conjunctivitis, a maculopapular rash starts on neck, behind ears, and
along hairline. Rash spreads downward and reaches feet in two to three days.

Erythema infectiosum (fifth disease):This is caused by parvovirus B19.

Frequently a low-grade fever with rash appearing 7 to 10 days later. Rash starts
as facial erythemathe slapped cheek appearance, then spreads to the trunk
and has an erythematous macular appearance. The rash often lasts longest on the

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extremities, where it has a lacy, reticular appearance.

Meningococcemia (Neisseria meningitides): Abrupt onset of rash, with fever,

chills, malaise, and prostration. Initial rash may be urticarial, maculopapular, or
petechial (marked by small, purplish hemorrhagic spots). In fulminant cases, can
become purpuric, with large hemorrhages into skin.

Roseola: Macular or maculopapular rash starts on trunk and spreads to arms and
neck. Rash preceded by three to four days of high fevers, which end as rash
appears. Usually in patients under two years of age.

Scarlet fever:Very fine erythematous, blanching papular rash, often described as

sandpaper-like. Starts in the groin, axillae, and neck but spreads rapidly. The
fever can be high, and the disease is usually self-limited, lasting less than 10
days. Etiologic agent is Group A streptococcus. Important to treat this to prevent
nonsuppurative complications of strep, including rheumatic fever.

Varicella: Rash starts on trunk and spreads to extremities and head. Each lesion
progresses from erythematous macule to papule to vesicle to pustule, and then
crusts over. Lesions at various stages of development. Self-limited disease lasting
approximately one week.

Physical exam:

Diffuse adenopathy: Can be seen with infectious mononucleosis

(Epstein-Barr virus or cytomegalovirus), human immunodeficiency virus
(HIV), histoplasmosis, toxoplasmosis, lymphomas, leukemia, histiocytosis,
metastatic neuroblastoma, and rhabdomyosarcoma. Measles also presents
with a marked generalized lymphadenopathy and splenomegaly that may
last several weeks.
Cervical adenopathy: Cervical adenopathy is common in children.
However, unilateral cervical adenopathy is significant. Possible causes
Bacterial cervical adenitis (most commonly due to Staphylococcus
aureus and Streptococcus pyogenes)
Cat scratch disease (Bartonella henselae infection); axillae most
commonly involved, but also cervical, submandibular, and inguinal
Reactive node from pharyngeal infection
Kawasaki disease
Mycobacterial infection (tuberculosis is most common cause in
children over 12 years)
Palmar rash: Rocky Mountain spotted fever, Kawasaki disease, syphilis
Strawberry tongue: Erythematous tongue with prominent papillae,
characteristic finding of both streptococcal pharyngitis and Kawasaki disease

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Differential diagnosis
1. Kawasaki disease: If patient febrile for five or more days, consider
Kawasaki disease. Other manifestations of this disease of unknown etiology
often elicited by history include a non-purulent conjunctivitis (which may
have cleared prior to presentation), rash, and swelling and erythema of the
hands and/or feet. The swelling in the feet can lead to painful walking. Must
have four of the following criteria in addition to fever (as well as no other
apparent cause for the illness) for diagnosis:
Changes in oral mucosa
Extremity changes (redness/swelling)
Unilateral cervical lymphadenopathy
2. Stevens-Johnson syndrome: Mucocutaneous disorder characterized by
fever, severe stomatitis, conjunctivitis, and erythema multiformetype (i.e.,
hypersensitivity reaction), blistering rash. Formerly called erythema
multiforme major.

3. Bone or joint infection:A concern in a young child who suddenly stops

walking, since up to 80% of infections are in the lower extremities.
However, the fever associated with osteomyelitis or septic arthritis is usually
not as high as in this case.

4. Juvenile idiopathic arthritis (JIA):A group of common rheumatic

diseases. Systemic-onset disease (Stills disease) characteristically has
prolonged fever, rash, and arthritis, although arthritis often presents much

5. Rocky Mountain spotted fever: Caused by Rickettsia rickettsii, this is the

most common fatal tick-borne disease in U.S. Characterized by fever,
myalgias, headache, and petechial rash classically starting on wrists and
ankles and progressing centrally.

6. Viral syndrome: A frequently used term covering wide range of illnesses.

The viruses most likely to cause fever, a non-descript rash, and presenting
in late summer/early fall would be the enteroviruses. The fever associated
with these viral infections in a normal host usually last no longer than three
to five days.

7. Scarlet fever: An erythematous, blanching, sandpaper-like rash with very

fine papules secondary to infection with Group A streptococcus. Starts in
groin, axilla, and neck. Spreads rapidly over trunk and extremities. Fever
can be high, but generally resolves within five days.


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Complete blood count (CBC) with differential:

Will help evaluate for possible infection or inflammatory response

Also useful to evaluate for possible anemia, thrombocytopenia, or
In Kawasaki disease, the white blood count is usually elevated, with a
predominance of neutrophils
A normochromic, normocytic anemia is common
A markedly elevated platelet count may be seen in the second week of the

Blood culture:

In this case, the clinical course is long for bacteremic illnesses, though the
height of the fever and the clinical appearance of the patient could justify
obtaining a culture.

Liver enzymes:

May be elevated in a number of diseases, including both Kawasaki disease

and Stevens-Johnson syndrome.
Serum albumin level frequently low in Kawasaki disease.

Acute phase reactants:

Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)

Lack of specificity limits usefulness for diagnostic purposes, though can lend
support for and against some diagnoses.
ESR and CRP frequently play a more useful role in following a disease
A normal ESR would argue strongly against Kawasaki disease.

Clean-catch urinalysis:

Sterile pyuria is associated with Kawasaki disease, secondary to a sterile

A clean-catch urine is likely to show white cells, whereas a catheterized
urine may not because the white cells come from the urethra.

Acute treatment of Kawasaki disease:

1. Hospitalization is required for treatment and evaluation of possible

2. Administration of high-dose intravenous immune globulin (IVIG), the only
known therapy to reduce the risk of coronary artery sequelae
Use of IVIG in Kawasaki disease has brought the incidence of coronary
artery aneurysms down from 2025% to 24%.
3. High-dose aspirin until defervescence

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Aspirin is used as an anti-inflammatory drug that helps shorten the

febrile course of the illness
Has no effect on the development of aneurysm
Other antipyretics usually not effective
Children on long-term aspirin therapy may be at risk for experiencing
Reye syndrome after influenza virus infection.
4. Echocardiography
All patients should receive an echocardiogram during the acute phase,
both to look for presence of coronary aneurysms and to provide a
baseline for future comparisons.
Aneurysms may be present by the end of the first week, but usually
present later, and almost always within four weeks of disease onset.

Prevention of complications:

Continue low-dose aspirin for at least six weeks following acute phase.
Since if coronary artery aneurysms develop they usually do so within four
weeks of the acute illness, follow-up visits with cardiology after discharge
for repeat echocardiograms are essential.
May require lifelong cardiology follow-up.

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