Kawasaki’s Disease

Pathophysiology

1) necrotizing vasculitis of early childhood 2) generalized rash (exantham) 3) fever 4) conjuncitivitis 5) lymphadenitis 6) oral lesions 7) possible infectious cause includes parvovirus B19

Signs and Symptoms

1) fever (always present) 2) skin rash 3) conjuncitivitis 4) lymphadenopathy 5) coronary artery aneurysms (25%) 6) cervical adenitis 7) strawberry tongue (also seen in scarlet fever) 8) crusting and fissuring of lips 9) erythema of hands and feet followed by desquamation 10) edema of mucous membranes

Characteristic Test Findings

Laboratory – 1) antiendothelial cell antibodies 2) increased IL-2 receptors 3) increased IL-1 receptors

Histology/Gross Pathology

1) infarcts and hemorrhage in heart and kidney 2) vasculitis of medium-size arteries

Associated Conditions

1) pericarditis 2) myocarditis 3) myocardial infarction 4) cardiomegaly 5) Staphylococcus aureus 6) toxic shock syndrome

Biochemistry

1) overstimulation of immune system is postulated 2) possibly caused by microbial stimulated production of superantigen to major histocompatiblity class 2 receptors and V-beta region of T cell receptor

Inheritance/Epidemiology

mortality is 3%

Treatment

1) generally unresponsive to antibiotics 2) aspirin 3) high dose gamma globulin

Tips for USMLE

1) clinically resembles scarlet fever and toxic shock syndrome 2) diagnosis – presence of fever ofr > 5 days and presence of four of the following: bilateral conjunctivitis, exanthem