Case 11

Question 1

What are key history findings for a patient with Kawasaki disease?

Answer 1

Persistent fever, irritability, rash, conjunctivitis, refusal to walk.

Question 2

What are key physical exam findings for a patient with Kawasaki disease?

Answer 2

Maculopapular rash, Nonpurulent conjunctivitis, Mucosal changes, Unilateral cervical adenopathy, Swelling in extremities.

Question 3

What is on the differential for Kawasaki disease?

Answer 3

Stevens-Johnson Syndrome, Juvenile rheumatoid arthritis, Measles, Kawasaki disease, Rocky Mountain spotted fever, Meningococcemia, Scarlet fever, Enterovirus.

Question 4

What are key lab findings for a patient with Kawasaki disease?

Answer 4

WBC elevated with neutrophilic predominance. Hemoglobin/hematocrit and red blood cell smear: Normochromic, normocytic anemia. Mean corpuscular volume: Normal. Platelets: Normal.

Question 5

Enterovirus exanthem:

Answer 5

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.

Question 6

Measles exanthem:

Answer 6

Initial rash appears on buccal mucosa as red lesions with central bluish white spots (Koplik spots). After prodromal fever (over 101 F), 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.

Question 7

Erythema infectiosum (fifth disease) exanthem:

Answer 7

This is caused by parvovirus B19. Frequently a low-grade fever with rash appearing 7 to 10 days later. Rash starts as facial erythema - the “slapped cheek” appearance, then spreads to the trunk and has an erythematous macular appearance. The rash often lasts longest on the extremities, where it has a lacy, reticular appearance.

Question 8

Meningococcemia (Neisseria meningitides) exanthem:

Answer 8

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.

Question 9

Roseola exanthem:

Answer 9

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 patents under two years of age.

Question 10

Scarlet fever exanthem:

Answer 10

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.

Question 11

Varicella exanthem:

Answer 11

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.

Question 12

Diffuse adenopathy:

Answer 12

Can be seen with infectious mononucleosis (EBV or CMV), HIV, histoplasmosis, toxoplasmosis, lymphomas, leukemia, histiocytosis, metastatic neuroblastoma, and rhabdomyosarcoma. Measles also presents with markedly generalized lymphadenopathy and splenomegaly that may last several weeks.

Question 13

Cervical adenopathy:

Answer 13

Cervical adenopathy is common in children. However, unilateral cervical adenopathy is significant. Possible causes include:

• Bacterial cervical adenines (most commonly due to Staph aureus and strep pyogenes)
• Cat scratch disease (bartonella henselae infection) axillae most commonly involved, but also cervical, submandibular and inguinal regions.
• Reactive node from pharyngeal infection
• Kawasaki disease
• Mycobacterial infection (tuberculosis is most common cause in children over 12 years)

Question 14

Palmar rash:

Answer 14

Rocky Mountain spotted fever, Kawasaki disease, Syphilis

Question 15

Strawberry tongue:

Answer 15

Erythematous tongue with prominent papillae, characteristic finding of both streptococcal pharyngitis and Kawasaki disease.

Question 16

What is on the differential diagnosis for Kawasaki disease?

Answer 16

Stevens-Johnson Syndrome, Bone or joint infection, Juvenile idiopathic arthritis (JIA), Rocky Mountain Spotted Fever, Viral syndrome, Scarlet fever.

Question 17

Kawasaki disease criteria:

Answer 17

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.

Question 18

Must have four of the following criteria in addition to fever (as well as no other apparent cause for the illness) for diagnosis:

Answer 18

• Changes in oral mucosa
• Extremity changes (redness/swelling)
• Unilateral cervical lymphadenopathy
• Rash
• Conjunctivitis

Question 19

Stevens-Johnson Syndrome:

Answer 19

Mucocutaneous disorder characterized by fever, severe stomatitis, conjunctivitis, and erythema multiforme-type (i.e., hypersensitivity reaction), blistering rash. Formerly called “erythema multiform major”

Question 20

Bone or joint infection:

Answer 20

A concern in a young child who suddenly stops walking, since up to 80 percent of infections are in the lower extremities. However, the fever associated with osteomyelitis or septic arthritis is usually not as high as this case.

Question 21

Juvenile idiopathic arthritis (JIA):

Answer 21

A group of common rheumatic diseases. Systemic-onset disease (Stills disease) characteristically has prolonged fever, rash and arthritis, although arthritis often presents much later.

Question 22

Rocky Mountain spotted fever:

Answer 22

Caused by rickettsia rickettsii, this is the most common fatal tick-borne disease in US. Characterized by fever, myalgias, headache, and petechial rash classically starting on wrists and ankles and progressing centrally.

Question 23

Viral syndrome:

Answer 23

A frequently used term covering a wide range of illnesses. The viruses most likely to cause fever, a nondescript 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.

Question 24

Scarlet fever:

Answer 24

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.

Question 25

CBC with differential in Kawasaki Disease:

Answer 25

• Will help eval. for possible infection or inflammatory response
• Also useful to evaluate for possible anemia, thrombocytopenia or thrombocytosis
• In Kawasaki disease, the WBC 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 illness

Question 26

Blood culture in Kawasaki disease:

Answer 26

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.

Question 27

Liver enzymes in Kawasaki disease:

Answer 27

• May be elevated in a number of disease, including both Kawasaki disease and Stevens-Johnson Syndrome
• Serum albumin level frequently low in Kawasaki disease.

Question 28

Acute phase reactants in Kawasaki disease:

Answer 28

• 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 process
• A normal ESR would argue strongly against Kawasaki disease

Question 29

Clean-catch urinalysis and Kawasaki disease:

Answer 29

• Sterile pyuria is associated with Kawasaki disease, secondary to a sterile urethritis.
• A clean-catch urine is likely to show white cells, whereas a catheterized urine may not because the while cells come from the urethra.

Question 30

Acute treatment of Kawasaki Disease:

Answer 30

1. Hospitalization is required for treatment and evaluation of possible complications.
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 to incidence of coronary artery aneurysms down from 20-25 percent to 2-4 percent.
3. High-dose aspirin until defervescence (dec. in fever)
   - 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 pts. should receive an ECHO 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.

Question 31

Prevention of Kawasaki Complications:

Answer 31

• Continue low-dose aspirin for at least 6 weeks following acute phase.
• Since if coronary artery aneurysms develop the usually do so within four weeks of the acute illness, follow-up visits with cardiology after discharge for repeat ECHOs are essential.
• May require lifelong cardiology follow-up