Answer to CC #13

Case challenge # 13 presented a 20-year-old white male with fever, conjunctival injection, cervical lymphadenopathy, cracked/red lips, a macular rash, swelling in his hands, a history of tonsillar abscess, and elevated inflammatory markers. Work-up for infectious disease and auto-immune disease are negative.

What is the most likely diagnosis?Screen Shot 2015-04-24 at 7.04.50 PM

The correct answer is Kawasaki Disease!
  • Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute necrotising vasculitis of the medium- and small-sized vessels. It was first described by Tomisaki Kawasaki in 1967. It occurs most often in babies and children, aged 6 months to 5 years and the male-to-female ratio ranges from 1.5–1.8 to 1. KD is most prevalent in Japan, while Korea holds the second place as to the number of patients. Its incidence in Japanese and Korean children living in the USA and following a Western lifestyle is higher than in Caucasian children. Since the disease is not common in adults, it is very often misdiagnosed. As of earlier this year, approximately 100 cases in the world population. 
  • Diagnostic Criteria: Fever persisting at least 5 days and the presence of at least 4 of the following 5 principal features:

    1. Changes in extremities: Acute: Erythema and edema of hands and feet Convalescent: Membranous desquamation of fingertips

    2. Polymorphous exanthema

    3. Bilateral, painless bulbar conjunctival injection without exudate

    4. Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae

    5. Cervical lymphadenopathy (≥1.5 cm in diameter), usually unilateral

    *Patients with fever and fewer than 4 principal symptoms can be diagnosed as having Kawasaki disease when coronary artery disease is detected by 2-dimensional echocardiography or coronary angiography. Other diagnoses should be excluded. The physician should be aware that some children with illness not fulfilling these criteria have developed coronary artery aneurysms.

Case challenge #14 (aka the Parkland Files!) will be posted next week!