Pathophysiology (Theoretical Based) Non-Modifiable Factors Age: < 5 years old Race: Asian (mostly in Japan/Korean/Chinese) Gender: Boys

> Girls (2:1) Genetics: ITPKC gene (inositol-trisphosphate 3-kinase C) Modifiable Factors Environment: Exposure to virus or bacteria

RACE Asian

AGE < 5 years old

GENDER Male

ENVIRONMENTAL Infection: bacterial/viral

GENETIC S ITPKC

inositol 1,4,5trisphosphate 3kinase C

Idiopathic

Impaired Immune System function

Exposure to bacteria and/or virus

Detection of foreign Body

Activation of Cell Immune Response

T-cells in thymus gland go in the lymph nodes

Widespread proliferation & activation of platelets

B-cells activation

Regulator T-cells in lymph nodes are stimulated

Effector T-cells in lymph nodes are stimulated

Spontaneous production of cytotoxic antibodies and immune complexes

Lymph nodes Lymphadenitis
Bilateral conjunctivitis, with or without pus or drainage Strawberry tongue
Red mucous membrane in mouth

Palpable lymph nodes on the cervical area Redness of the eyes

Cytotoxic T-cell production Suppresor T-cells No control of B-cell activity

Helper T-cells activation

Eyes

Mucosa

Finger gangrene

Attracts and activates more B-cells, cytotoxic Tcells, Natural killer cells, Macrophages and other cells in immune system

Lymphokine Production

General Circulation of body

Attacks and localizes on the system of the body

Skin

Lysis of normal cells in the skin

Desquamation or peeling of the skin, beginning on the nails

Red palms and soles of the feet Non-vesicle-bullous, non-itchy, non-specific, polymorphic rash in the trunk
Musculoskeletal

Attracts other lymphocytes and WBC Release of multitude of proinflammatory cytokines

Polyarthritis Inflammation of arteries

(+) Pain
Aneurysm Stagnant blood flow, activated platelets, inflammed endothelium

CVS

Thrombotic occlusion

Localized stenosis

MI, Embolism