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Start free with EleplanKawasaki disease
ORPHA:2331Disease
Also called Mucocutaneous lymph node syndrome
What it is
A rare inflammatory disease characterized by an acute febrile, systemic, self-limiting, medium-vessel vasculitis primarily affecting children. It often causes acute coronary arteritis which is associated with coronary arterial aneurysms (CAA) that may be life threatening when untreated.
Key facts
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Inheritance
- Multigenic/multifactorial
- Classified as
- Disease
Signs and symptoms
Very common80–99%
8- Cervical lymphadenopathyDiagnostic criterion
- CheilitisDiagnostic criterion
- ConjunctivitisDiagnostic criterion
- Palmoplantar erythemaDiagnostic criterion
- Proteinuria
- Recurrent pharyngitis
- Skin rashDiagnostic criterion
- Vasculitis
Common30–79%
21- Abdominal pain
- Abnormal heart valve morphology
- Abnormality of nail color
- Arthritis
- Conjunctival hyperemiaDiagnostic criterion
- Diarrhea
- Edema
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- Fatigue
- FeverDiagnostic criterion
- GlossitisDiagnostic criterion
- Hypoalbuminemia
- Increased total leukocyte count
- Lip fissureDiagnostic criterion
- Palmar edemaDiagnostic criterion
- Pericarditis
- Plantar edemaDiagnostic criterion
- Scaling skin on fingertipDiagnostic criterion
- Strawberry tongueDiagnostic criterion
- Thrombocytosis
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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