Kawasaki Disease

Question 1

Age of onset

Gender

Answer 1

Peak incidence at 1year,mean 2.6years,uncommon after 8years

Male

Japanese

Question 2

3 Phases.

Answer 2

PHASEI: ACUTE FEBRILE PERIOD - Abrupt onset of fever, lasting approximately 12 days,followed
(usuallywithin 1to3 days)by most of the other principal features. Constitutional symptoms of diarrhea, arthritis, and photophobia.

PHASEII:SUBACUTEPHASE - Lasts approximately until day 30 of illness; fever, thrombocytosis, desquamation, arthritis, arthralgia, and carditis; highest risk or sudden death.

PHASEIII:CONVALESCENT PERIOD Begins within 8 to 10 weeks after onset of illness when all signs of illness have disappeared and ends when ESR returns to normal.

Question 3

Skin lesions per phase

Answer 3

PHASEI Lesions appear 1 to 3 days after onset of fever. Duration, 12 days average. Nearly all mucocutaneous abnormalities occur during this phase.
Erythema usually first noted
on the palms and soles, spreading to involve trunk and extremities within 2 days. First lesions: Erythematous macules; lesions enlarge and become more numerous. type: Urticaria- like lesions (most common); morbilliform pattern (common); scarlatini form and EM like in<5%o cases.
Confluent macules to plaque- type erythema on perineum, which persist
after other findings have resolved. Edema o hands/ feet:Deeply erythematous to violaceous; brawny swelling with fusiform fingers. Palpation: Lesions may be tender. Mucous Membranes. Bulbar conjunctival injection;noted 2 days after onset of fever; duration, 1 to 3 weeks (throughout the ebrile course). Lips: Red, dry, ssured
hemorrhagic crusts; duration, 1 to 3 weeks. Oropharynx: Diffuse erythema. tongue: “Strawberry” tongue (erythema and protuber- anceo papillaeo tongue).
Cervical Lymphnodes. Lymphadenopathy tender, rm, > 1.5 cm.

PHASEII Desquamation highly characteristic;
follows resolution of exanthem. Begins on the tips of fingers and toes at the junctional nails and skin;desquamating sheet so palmar/plantar epidermis are progressively shed.

PHASEIII Beau lines (transverse urrows on nail sur ace) may be seen by Possible telogen ef uvium

Question 4

Lab tests.

Answer 4

CHEMISTRY Abnormal liver unction tests. 
HEMATOLOGY Leukocytosis (> 18,000/μL).
Thrombocytosis after the 10th day of illness. ElevatedESR in phaseII.
ESR  returns  to normal in phase III.
URINALYSIS Pyuria.

DERMATOPATHOLOGY Arteritis involving
small- and medium-sized vessels with swelling of endothelial cells in post capillary venules, dilatation of small blood vessels, lymphocytic/ monocytic perivascular in ltrate in arteries/ arterioles of dermis.

ELECTROCARDIOGRAPHY Prolongation of PR and QT intervals; ST segment and T -wave changes.

ECHOCARDIOGRAPHY AND ANGIOGRAPHY Coronary aneurysms in 20% o cases.

Question 5

Diagnostic criteria

Answer 5

Fever spiking to > 39.4°C, lasting ≥ 5 days without other cause, associated with our o ve criteria: (1) bilateral conjunc- tivalinjection;(2)atleastoneo the ollowing mucousmembranechanges—injected/ ssured lips, injected pharynx, “strawberry” tongue; (3) at least one o the ollowing extremity changes—erythema o palms/soles, edema o hands/ eet, generalized/periungual desqua- mation;(4)di usescarlatiniormordeeply erythematous maculopapular rash, iris lesion

Question 6

Management

Answer 6

HOSPITALIZATION Recommended during the phase I illness, monitoring or cardiac and vascular complications.
SYSTEMICTHERAPY
Intravenous Immunoglobulin. 2 g/kg as a single in fusion over 10 h together with aspirin as soon as possible.
Aspirin. 100 mg/kg per day until ever resolves or until day14 of illness, followed by
5 to 10 mg/kg per day until ESR and platelet count have returned to normal.

Glucocorticoids are Contraindicated. Associ ated with a higher rate of coronaryaneurysms