Clinical Challenge: Fever and Rash for Eight Days


  • Kawasaki disease desquamation_1211 Derm dx

  • Kawasaki disease desquamation perineum_1211 Derm D

  • Kawaski disease skin_1211 Derm Dx

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  • Kawasaki disease lips_1211 Derm Dx

  • Kawasaki disease tongue_1211 Derm Dx

A 2-year-old black female presents to the ED with her mother after experiencing eight days of high-grade fever (up to 103° F) and six days of rash. The mother states that the rash began on the patient’s hands and feet and subsequently became generalized.

On day three of illness, the patient developed erythema in her perineum, which start to peel and desquamate on day five. The mother states that on the fourth febrile day, her daughter’s lips became very cracked and started to bleed on day five.

On physical exam, the patient appears fussy and uncomfortable. She is febrile at 102°F, and her other vital signs are within normal limits. Her trunk and extremities are covered with multiple erythematous macules and papules, coalescing into patches and plaques associated with multiple pinpoint pustules.

In the patient’s perineum there is evidence of complete superficial desquamation, and she has shotty cervical lymphadenopathy. The hands and feet are mildly edematous, and her lips are fissured and covered with extensive hemorrhagic crust. The tongue papilla are red and inflamed giving the appearance of a strawberry. There is mild bilateral conjunctival injection. The oral, vaginal, and anorectal mucosa are without erosions or ulcerations.

Laboratory studies show an elevated erythrocyte sedimentation rate (ESR), and a moderate thrombocytosis and leukocytosis. Blood and urine cultures are negative.

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Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an idiopathic febrile syndrome that primarily affects children younger than five years. The main source of morbidity and mortality in KD is coronary artery aneurysms, which occur in 20% to...

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Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an idiopathic febrile syndrome that primarily affects children younger than five years. The main source of morbidity and mortality in KD is coronary artery aneurysms, which occur in 20% to 25% of untreated patients.

KD is in fact the most common cause of acquired heart disease in developed countries, including the United States. With treatment, the risk of coronary artery aneurysm disease is significantly reduced to 3% to 5%. Therefore, it is important that this syndrome is recognized and treated early. 

Clinical findings include fever, skin eruption, cervical lymphadenitis, edema of the hands and feet, conjunctival injection and oropharyngeal changes (strawberry tongue, diffuse hyperemia and lip fissures). Fever is usually greater than 102° F and must be present for greater than five days to meet KD diagnosis criteria.

The skin eruption is polymorphous and may be morbilliform, pustular, urticarial, scarlatinaform or erythema-multiforme like. Perianal and perineal erythema with subsequent desquamation within the first week is quite characteristic.

One to two weeks after illness onset, the fingers and toes desquamate, starting around the nails.  Coronary arterial disease begins after day 10 and can lead to cardiac events.  Laboratory abnormalities may include thrombocytopenia during the first week with a subsequent thrombocytosis, leukocytosis, elevated inflammatory markers such as c-reactive protein and ESR, sterile pyuria, anemia and a hepatic transaminitis. 


KD is a clinical diagnosis. The diagnostic criteria include fever lasting for more than five days, plus four out of the five following criteria:

  • Skin eruption (described above)
  • Oropharyngeal changes (described above)
  • Bilateral conjunctival injection
  • Cervical lymphadenopathy
  • Edema of the hands and feet (with subsequent desquamation)

Incomplete KD describes a syndrome in which the patient has fever but does not have four out of the five above criteria. Incomplete KD is more common in infants. Because these patients are still at risk for coronary artery disease, a high level of clinical suspicion is necessary to initiate therapy promptly to prevent cardiac disease. 


Treatment includes high dose aspirin therapy and a single dose of intravenous immunoglobulin G (IVIG).  IVIG is administered in one single dose of 2 mg/kg during a 10 to 12 hour infusion period. 

Aspirin is initiated at 80 to 100 mg/kg daily in four divided doses until the patient is afebrile for 48 to 72 hours. The aspirin is subsequently tapered to 3 to 5 mg/kg in one daily dose for six to eight weeks.  Echocardiogram and EKG and long term cardiac follow-up are mandatory. 

Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine in Houston.


1. Bolognia, J, Jorizzo JL, Rapini RP. “Chapter 112: Benign Melanocytic Neoplasms.” Dermatology. 2008: Mosby/Elsevier; St. Louis, Mo.

2. James WD, Berger TJ, Elston DM, and Odom RB. “Chapter 80: Other Viral Disease.” Andrews’ Diseases of the Skin: Clinical Dermatology. 2006: Saunders Elsevier; Philadelphia.